interactive case 4 cardiac b

Section 3D-4Sabalvaro, D.K., Salac, C.N., Salazar, J., Salazar, R., Salcedo, V.E., Saldana, E., Sales, M.S.A., Salonga, C.A., San Diego, P., San Pedro, R.*

To present a case of a 2 year old blue baby To give the clinical impression and differential diagnosis of the case based on the history and physical examination To present the Chest X ray of the patient To discuss the pathophysiology and symptomatology of Tetralogy of Fallot To discuss other diagnostic procedures that may be useful in TOF To discuss the management and prognosis of a patient with TOFOBJECTIVES*

GENERAL DATAB.B.3 year old maleblue baby

Chief complaint: progressive cyanosis *

HISTORY OF PRESENT ILLNESSBorn blue babyUnspecified heart disease

1 week PTA More bluish lips (+) easy fatigability (+) seen to sit down after a short walk

Few hours PTA While feeding, patient developed bluish hue of the face and extremities

ADMISSION*

REVIEW OF SYSTEMSPoor appetite, poor weight gainNo skin lesions; cyanotic since birthShortness of breath on exertionBluish episodes while feeding

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PAST MEDICAL HISTORYBorn a blue babyPoor birth weight, retarded growthOccasional visit to ER due to cyanosis when crying(-) Asthma/allergies(-) Previous surgeries

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FAMILY HISTORY(-) Heart disease(-) Diabetes(-) Asthma/allergies(-) PTB*

PERTINENT PHYSICAL EXAMINATIONConscious, agitatedBP 90/60, HR 90 bpm, RR 30s, small for ageWarm moist skin, no dermatosesBluish lip and buccal ,mucosaHeart: (+) thrill along left sternal border(+) harsh systolic murmurSingle S2Extremities: bluish nail beds, (+) clubbing*

MISSING DATA

Obstetrical History: Illnesses of mother during gestationEpisodes of tet spells - marked increase in cyanosis followed by syncopeSquatting (?)*

SALIENT FEATURES3 y/o MaleCyanotic since birthDifficulty in feeding, poor birth weight, retarded growth(+) dyspnea and easy fatigability(+) clubbing, cyanosis of the lips, buccal mucosa and nail bedsHeart: (+) thrill along left sternal border (+) harsh systolic murmur single S2*

CLINICAL IMPRESSION

Congenital Cyanotic Heart Disease, probably Tetralogy of Fallot*

Reference: http://www.bcm.edu/radiology/cases/pediatric/text/2i1A.htm*

Congenital AnomalyPathologySigns and SymptomsChest RadiographTransposition of Great VesselsTwo major arteries leaving the heart are connected to the wrong ventricles or lower chambers of the heart.Blue or cyanotic shortly after birth. The blueness doesn't go away even if the baby is given extra oxygen.Shortness of breath Poor feeding Clubbing of the fingers or toes Increased vascularityCardiomegalyCardiac silhouette: egg on its side; apple on a stem Narrow vascular pedicleTotal Anomalous Pulmonary Venous ReturnNone of the pulmonary veins are connected to the left atrium but instead all drain by various routes into the right atrium.

Type1: supracardiacType2: intracardiacType3: infracardiacCyanosis and respiratory distress Cyanosis made worse on feeding, especially with the infracardiac type caused by the compression of the common pulmonary vein by the food-filled esophagus. (-) Cardiac murmur Increased pulmonary vascular markings which, if untreated, progress to cardiac failure. A 'snowman' sign or figure of 8 configuration in the supracardiac type but rarely before 4 months of age.

References: http://emedicine.medscape.com/article/158359-diagnosis and http://www.bcm.edu/radiology/cases/pediatric/text/2h-desc.htm*

Congenital AnomalyPathologySigns and SymptomsChest RadiographTruncus Arteriosus Only one artery connects to the heart instead of two.Large VSD over which a large, single great vessel (truncus) arises. This single great vessel carries blood both to the body and to lungs. Blood from both ventricles mixes together as it all exits through the single valve exiting from the heart. Bluish cast to their skin, especially around the nose and mouth. BreathlessnessRapid breathingExcessive sweatingRestlessness Increased vascularityPulmonary venous edemaCardiomegalyLeft atrium and either/both ventricles prominenceConcave main pulmonary artery segmentLarge aortic shadowRight aortic arch in 35%Tricuspid AtresiaComplete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle.Congestive heart failureGrowth retardationPoor skin coloration (pallor to frank cyanosis)Inability to complete a feeding sessionFrequent pauses during feeding, and/or frank anorexiaRespiratory difficulties reported as nasal flaring or muscle retractions, digital clubbingCardiomegaly with a prominent right heart border reflecting RA enlargementDiminished pulmonary vascular markingsA right aortic arch in 3-8% of cases

Reference: http://emedicine.medscape.com/article/154447-diagnosis*

Congenital AnomalyPathologySigns and SymptomsChest RadiographHypoplastic Left Heart SyndromeMarked hypoplasia of the left ventricle and ascending aortaatretic, hypoplastic or stenotic aortic and mitral valvesIntact ventricular septumPDA supplies blood to the systemic circulation. (+) ASD or patent foramen ovaleCoexisting coarctation of the aortaRespiratory symptoms and profound systemic cyanosis at birthAs the ductus arteriosus begins to close normally over the first 24-48 hours of life, symptoms of cyanosis, tachypnea, respiratory distress, pallor, lethargy, metabolic acidosis, and oliguria develop. No intervention to reopen the ductus arteriosus deathChest radiographic findings are not specific for hypoplastic left heart syndrome.Cardiomegaly and increased pulmonary venovascular markings are typically present.Marked pulmonary edema may be noted in infants with obstructed pulmonary venous return.

Ebsteins AnomalyApical displacement of the septal and posterior tricuspid valve leaflets atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet.FatigueDyspnea Palpitations Clubbing Precordial asymmetryAnkle edema Ascites (Symptoms of right heart failure)Normal findingsCardiomegalySmall aortic root and main pulmonary artery shadowDecreased pulmonary vasculatureLarge right atrium

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Congenital AnomalyPathologySigns and SymptomsChest RadiographTetralogy of FallotMaldevelopment of RV infundibulumPulmonary artery stenosis VSDDeviation of the aortic origin to the rightRVHThe degree of right ventricular outflow tract obstruction accounts for the deleterious hemodynamics of TOF.Poor feeding, cyanosis during feeding, fussiness, tachypnea, and agitationDyspnea on exertionSquattingLow birthweight Growth retardationDelayed development and pubertyCoeur en sabot secondary to uplifting of the cardiac apex from RVH and the absence of a normal main pulmonary artery segmentDecreased pulmonary vascularityRA enlargementRight-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow

NORMAL CHEST X RAY OF A 3-YEAR OLD BABY123456789

TracheaRightatriumBronchialbifurcationCardiophrenicangleCostophrenicangleNORMAL CHEST X RAY OF A 3-YEAR OLD BABY

NORMAL CHEST X RAYCARDIO-THORACIC RATIO and DIAPHRAGMSA/B 0.65AB

NORMAL CHEST X RAY: LATERAL VIEW

PA VIEWPATIENTS CHEST X RAYLATERAL VIEW

PA VIEWPATIENTS CHEST X RAYABCT RATIO = 0.66Decreased pulmonary vasculature

Cardiac apex displaced upward coer en sabot

Lifted up ApexPATIENTS CHEST X RAY

Concave MPARPAR Aortic ArchRight ventricular hypertrophyPATIENTS CHEST X RAY

PATIENTS LATERAL VIEW

Tetralogy of Fallot4 Features:Ventricular Septal DefectObstruction of the right ventricular outflow tractOverriding aortaRight Ventricular hypertrophy*

SymptomatologyEach child can present with different symptoms which depend upon the severity of the obstruction and stenosis as seen in the pathophysiology of the disease.

Blue color of the skin, lips and nail beds (central and peripheral cyanosis) which occurs with crying or feeding.

Some babies do not have noticeable cyanosis, but may instead be very irritable or lethargic due to a decreasing amount of oxygen available in the bloodstream due to admixing of oxygenated and less oxygenated blood.

Some children may have pale or ashen color and may have cool clammy skin.

Dyspnea and syncope

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Physical Exam FindingsRV predominance on palpationMay have a bulging left hemithoraxSystolic thrill at the lower left sternal borderAortic ejection clickSingle S2 - Pulmonic valve closure not heardSystolic ejection murmur - varies in intensity inversely with the degree of RVOT obstructionMore cyanotic patients have greater obstruction and a softer murmur.An acyanotic patient with TOF has a long, loud, systolic murmur with a thrill along the RVOT.Cyanosis and clubbing - variableSquatting position- compensatoryScoliosis - commonRetinal engorgementHemoptysis

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PathophysiologyResult from anterosuperior displacement of the infundibular septumSeverity of obstruction to RV outflow determines the direction of blood flow.If subpulmonary stenosis is mild- shunt, may be left-to-right w/o cyanosis. in severity of obstruction = in resistance to RV outflow, right-to-left shunting predominates along with cyanosis subpulmonic stenosis, pulmonary arteries are smaller and hypoplastic and the diameter of the aorta becomes progressively larger.RVH - compensatoryAs the child grows, the pulmonic orifice does not expand proportionally to the enlarging heart, making the obstruction worse.

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OTHER DIAGNOSTIC PROCEDURESUltrasonographyAngiographyCT ScanMRI

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UltrasonographyFindings:Echocardiography is the primary imaging method for examining a child in whom TOF is suspected. Intracardiac anomalies, including pulmonary infundibular and valvular stenosis and the position of the aortic root overriding the ventricular septal defect, are identified with 2-dimensional echocardiography. The origins of the coronary arteries can also be identified.

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UltrasonographyDoppler ultrasonographic examination of the pulmonary outflow tract can be used to measure the velocity gradient in the right ventricular outflow tract and to differentiate severe stenosis from atresia. Continuity of the branch pulmonary artery with the main pulmonary artery can be identified, and the size of the branch pulmonary arteries can be measured. *

UltrasonographyThe initial placement of palliative shunts is likely in childrenwho havesmall branch pulmonary arteries in order to allow the pulmonary arteries to growbefore corrective surgery. The full length of the shunts may not be visible; however, Doppler ultrasonography can be used to verify shunt patency, even when the entire length of the shunt cannot be imaged.

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Computed TomographyInfrequent role in the evaluation of TOFUseful for the evaluation of surgical complications such as infection or pseudoaneurysm formation. Helical CT scanning can be used to identify airway compression that is caused by a large ascending aorta that is associated with TOF.

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Magnetic Resonance ImagingSpin-echo MRI can be used to identify the morphologic abnormalities of TOF, which are as follows: Right ventricular outflow tract obstructionVSDRVHOverriding aorta*

NORMAL MRI Right ventricularcavityPulmonary arteryPericardiumRight ventricular myocardiumLeft ventricularcavityLeft ventricular myocardiumInterventricularseptum*

NORMAL MRI Right lungAscending aortaRight ventricular cavityAortic valveLeft ventricularcavityPulmonary artery*

NORMAL MRI Brachocephalic arteryLeft brachiocephalicveinLeft pulmonaryarteryAortaRight lungRight pulmonaryarteryRight ventricularcavityLeft commoncarotid arteryLeft ventricularcavity*

NORMAL MRI Right brachiocephalicveinLeft pulmonaryarteryAortic archRight lungSuperior vena cavaRight atriumTracheaLeft atriumRight pulmonaryarteryHepatic vein*

Overriding aortaHigh VSD

TOF in MRI*

Magnetic Resonance ImagingThe confluence, presence, and size of the branch pulmonary arteries can be identified. MRI measurements of the size of the pulmonary and branch pulmonary arteries are as accurate as angiographic measurements, and they can be used to calculate the McGoon ratio and the Nakata index ( Branch pulmonary arteries).

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Magnetic Resonance ImagingPostoperative evaluation of pulmonary artery stenoses is better with MRI than with echocardiography. Cine imaging can be used to identify pulmonary stenosis or regurgitation, which is depicted as flow voids. RV enlargement is best quantified with MRI. Flow-analysis quantification of pulmonary regurgitation is unique to MRI. Although gradients can be measured with echocardiography, only MR flow analysis enables the accurate cross-sectional measurement of flow.

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AngiographyGold standard in evaluating blood vessels

FindingsAngiography is the traditional criterion standard and best modality for the evaluation of thepulmonary and coronary arterial morphology, as well as the morphology of the systemic collateral arteries.

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Angiography The branch pulmonary arteries have a characteristic seagull appearance. Pulmonary arterial measurements for the calculation of the McGoon ratio and Nakata index are critical to surgical planning. An aortic root injection is used to evaluate the position and number of coronary arteries.*

TOF in AngiogramLPA StumpMPACatheterDescending branch of RPARPAAscending AortaAortic ArchDescending Aorta*

To present a case of a 2 year old blue baby To give the clinical impression and differential diagnosis of the case based on the history and physical examination To present the chest xray of the patient To discuss the pathophysiology and symptomatology of Tetralogy of Fallot To discuss other diagnostic procedures that may be useful in TOF To discuss the management and prognosis of a patient with TOFOBJECTIVES*

ManagementTreatment depends on the severity of right ventricular outflow tract obstruction.Immediate increase in pulmonary blood flow to prevent hypoxia.Oxygenation, normal body temperature, normal glucose level should be maintained.Prevention and prompt treatment of dehydration to prevent hemoconcentration and possible thrombotic episodes.*

ManagementFor Tet spellsPropanolol (0.51 mg/kg every 6 hr)Morphineto reduce ventilatory drive Vasopressor such asepinephrine to increase blood pressureModified Blalock-Taussig shunt Aortopulmonary shunt *

PrognosisUNTREATED:Untreated TOF may lead to further RV hypertrophy and dilated cardiomyopathy (beginning on the right,then left).Actuarial survival for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years.*

PrognosisTREATED:After successful total correction, patients are generally asymptomatic and are able to lead unrestricted lives.Have lower than normal exercise capacity, maximal heart rate and cardiac output (more frequent in those who have undergone surgery at a later age).Uncommon immediate postoperative problems include right ventricular failure, transient heart block, residual VSD with left-to-right shunting, and myocardial infarction from interruption of an aberrant coronary artery.A number of children have premature ventricular beats after repair of TOF.Lifetime follow-up care*

REFERENCESMisra, Rakesh, et. al. A-Z of Chest Radiology, 2007.Novelline, Robert A. Squires Fundamentals of Radiology, 6th ed.Sutton. A Textbook of Radiology and Imaging.http://emedicine.medscape.com/article/158359-diagnosishttp://www.bcm.edu/radiology/cases/pediatric/text/2i1A.htmhttp://www.bcm.edu/radiology/cases/pediatric/text/2h-desc.htm

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***************ToGV Corrected transposition/ventricular inversion, L-TGACongenitally corrected transposition of the great arteries also termed ventricular inversion/ L-TGA, is characterized by atrioventicular discordance and ventriculoarterial discordance, hence the left atrium connects to the right ventricle which connects to the aorta. Congenitally corrected transposition accounts for 0.5% of congenital heart defects.Embryology: Normally related cardiac connections arise from d-looping of the embryonic heart tube. L-looping of the embryonic tube results in the heart tube looping to the left while all other lateralization remains the same i.e. atrial situs solitus.Associated lesions: (1)Ventricular septal defect occurs in approximately 80% of lesions. This may be single or multiple. The majority of patients with ventricular septal defects have associated pulmonary stenosis(PS). (2) PS occurs in some 50% of patients and is usually subvalvular stenosis secondary to bulging of the malaligned ventricular septum. (3) Atrioventricular valve abnormalities occur in up to 90% of patients. This may represent Ebsteins anomaly with severe displacement of the tricuspid valve into the right ventricle or mild elongation or redundancy of the leaflets. Straddlingof the ventricular septum by either AV valve may also occur particularly in association with an inlet ventricular septal defect. (4) Conduction abnormalities including complete AV block occur in up to 30% of patients. First or second degree heart block in 20-30%. The prevalence of complete AV block increases with age. (5) Dextrocardia occurs in one quarter of patients with ventricular inversion/L-TGA. (6) Left superior vena cava to coronary sinus.CXR: Characteristically, in patients with ventricular inversion and transposition of the great arteries, the upper left heart border is straightened as a result of leftward displacement of the aorta.ECG: Typically, there is reversed septal depolarization with absent Q waves over the left chest in association with some degree of AV block.PA chest radiograph shows cardiomegaly with increased pulmonary vascular markings secondary to a ventricular septal defect. The right pulmonary artery appears to have a high take-off because of an absent aortic shadow and is also quite prominent indicating ventricular inversion, L-TGA.The heart is enlarged with a narrow "pedicle" giving the so called "egg on a string" appearance. The superior mediastinum appears narrow due to the antero-posterior relationship of the transposed great vessels and "radiologic-absence of the thymus".

Total anomalous pulmonary venous connection (TAPVC)Total anomalous pulmonary venous connection is preferable to anomalous venous return as one may have anomalous drainage in the absence of anomalous connection. Winslow reported the first documented case of PAPVC in 1739 in a patient with a right upper pulmonary vein draining to the superior vena cava.Anomalous pulmonary venous connection is classified as either partial or total. In TAPVC all the pulmonary veins drain into the right atrium either directly or via a venous channel. In all cases there is an ASD or patent foramen ovale which allows right to left atrial shunting in order to maintain survival. Approximately 1/3 of patients with TAPVC have other associated cardiac lesions including single ventricle, atrioventricular septal defect, transposition of the great arteries, hypoplastic left heart syndrome or patent ductus arteriosus. Many of these patients have heterotaxy syndrome with atrio-visceral situs abnormalities and polysplenia/asplenia (Ivemark syndrome).Incidence: TAPVC accounts for less than 1% of all cardiac defects. There is a 3:1 male preponderance in infants with infradiaphragmatic TAPVC.Classification: Type 1: Supracardiac connection (55%); connection to the left innominate vein is the commonest accounting for some 44% of all TAPVC. Typically two anomalous veins from each lung converge directly behind the left atrium and form a common anomalous vertical vein, which passes anterior to the left pulmonary artery and the left main bronchus. Obstruction in this lesion is uncommon. However extrinsic compression may occur in cases where the anomalous vein courses between the left pulmonary artery anteriorly and the left main bronchus posteriorly. Anomalous connection to the right superior vena cava is much less frequent but often associated with heterotaxy syndrome/ multiple complex congenital lesions. Type 2: Cardiac connecion (30%); the pulmonary veins connect at the level of the coronary sinus or in the posterior right atrium near the mid-atrial septum. The anomalous veins may connect via a short channel or multiple openings to the right atrium. The coronary sinus ostium is markedly enlarged although normal in position. One paper reported a 22% incidence of obstruction in this lesion. Type 3: Infracardiac connection (13%); this lesion is virtually always accompanied by some degree of obstructed venous return. The pulmonary veins from both sides converge behind the left atrium and form a common vertical descending vein, which courses anterior to the esophagus and traverses the diaphragm at the esophageal hiatus. This vertical vein may join the portal venous system (80-90% cases) either at the splenic or splenic-superior mesenteric venous confluence. Occasionally the vertical vein may connect directly to the ductus venosus or even the hepatic or inferior vena cava. Obstruction to venous drainage may obviously occur at any point along the abberant path including the esophageal hiatus, the portal venous system or the ductus venosus (discrete). Obstruction may also occur at the level of the hepatic sinusoids (diffuse obstruction). Presentation is generally in the early newborn period. Type 4: Mixed pattern (2%); the commonest pattern of mixed obstruction is drainage of a vertical vein to the left innominate vein and drainage of the right lung either via the right atrium or the coronary sinus. This pattern of anomalous venous connection is generally associated with other major cadiac lesions.

Radiograph:type1 supracardiac: There is cardiomegaly with increased pulmonary arterial markings. There is dilation of both the left and right innominate veins and the right superior vena cava producing the classical "snowman" or "figure of 8" appearance. The superior mediastinum is enlarged secondary to dilation of the right vena cava, innominate vein and ascending vertical vein.Infradiaphragmatic obstructed: PA chest radiograph demonstrates increased pulmonary venous pattern with a normal sized heart. There is a right sided pleural effusion. The endotracheal tube is just above the level of the carina.

The snowman configuration is due to widening of the superior mediastinum and cardiac silhouette.

*Truncus arteriosus - Babies with truncus arteriosus usually don't get enough oxygen in their blood so they may have a bluish cast to their skin, especially around the nose and mouth.Congestive heart failure is when one or more chambers of the heart fail to keep up with the volume of blood flowing through them. Chest radiograph: There is marked increase in pulmonary vascular markings as pulmonary vascular resistance has fallen. The superior mediastinum is widened due to large aortic shadow.This lesion was first described by Wilson in 1798. It was Lev and Saphir who provided the first definition in 1942 of truncus arteriosus as a single trunk leaving the heart, which gives rise to the aorta, pulmonary arteries and coronary arteries. Truncus occurs in approximately 1-4% of patients with congenital heart disease. Without surgical treatment mortality is 80% by one year.Anatomy: Truncus is characterized by a single large vessel which overrides an outlet perimembranous VSD and gives rise to the aorta, pulmonary arteries and coronary arteries. The VSD is usually leftward and anterior, in the supracristal position or within the crista.Classification: Collett and Edwards Type I: a short main pulmonary artery segment arise from the aorta which then branches into both pulmonary arteries.Type II: both branch pulmonary arteries arise from the common arterial trunk close to each other usually with a rim of truncal tissue between them. Type III: the branch pulmonary arteries arise from either side of the truncus and are remote from each other.Type IV: this is considered a form of pulmonary atresia, with collateral vessels arising from the descending aorta supplying the lungs.The truncal valve leaflets are often thickened secondary to expansion of the spongiosa and fibrosa layers of the leaflets. There may be 2-6 truncal valve leaflets. The incidence of truncal valve stenosis is 30% with truncal insufficiency reported in up to 50% of cases. The coronary arteries are often abnormal with the left often arising from a higher and more posterior position and occasionally the presence of a single coronary artery.Associated lesions: The most frequent anomalies are a right sided aortic arch (1/3 cases), an interrupted aortic arch (19%)- type B between the left carotid and left subclavian arteries being the commonest.

*Hypoplastic Left Heart Syndrome Although hypoplastic left heart syndrome can easily be detected on fetal echocardiography, many infants are not identified prenatally because routine obstetric ultrasound examination may not concentrate on cardiac anatomy. Pregnancies are typically uncomplicated, and fetal echocardiography is not indicated routinely. The fetus grows and develops normally because the fetal circulation is not altered significantly. Most neonates are born at term and initially appear normal.Occasionally, respiratory symptoms and profound systemic cyanosis are apparent at birth (2-5% of cases). In these infants, significant obstruction to pulmonary venous return (a congenitally small or absent patent foramen ovale) is usually present. As the ductus arteriosus begins to close normally over the first 24-48 hours of life, symptoms of cyanosis, tachypnea, respiratory distress, pallor, lethargy, metabolic acidosis, and oliguria develop. Without intervention to reopen the ductus arteriosus, death rapidly ensues.Cardiac examination Palpable right ventricular impulse Normal first heart sound Loud single second heart sound Nonspecific, soft, systolic ejection murmur at the left sternal border (not always present) High-pitched holosystolic murmur at the lower left sternal border, indicating tricuspid regurgitation (not always present) Diastolic flow rumble over the precordium, indicating increased right ventricular diastolic filling (not always present)Ebsteins AnomalyHistoryPatients can have a variety of symptoms related to the anatomical abnormalities of Ebstein anomaly and their hemodynamic effects or associated structural and conduction system disease. Cyanosis Fairly common and frequently due to right-to-left shunt at the atrial level and/or severe heart failure Transient in neonatal life with recurrence in adult life May appear for the first time in adult life Transient appearance/worsening of cyanosis in adult life due to paroxysmal arrhythmias Once apparent, progressively worsensFatigue and dyspnea: These are due to poor cardiac output secondary to right ventricular failure and decreased left ventricular ejection fraction. Palpitations and sudden cardiac death Due to paroxysmal supraventricular tachycardia (SVT) in as many as one third of patients Fatal ventricular arrhythmias, which may be due to the presence of accessory pathwaysSymptoms of right heart failure: These include ankle edema and ascites. Other less common presenting symptoms Brain abscess due to right-to-left shunt Bacterial endocarditis Paradoxical embolism, stroke, and transient ischemic attacksPhysicalPhysical findings, like the symptoms, span a spectrum from subtle to dramatic.Cyanosis and clubbing - Varying degrees of cyanosis at various times in life and transient worsening with arrhythmias Precordial asymmetry Usually left parasternal prominence and occasionally right parasternal prominence Absent left parasternal (ie, right ventricular) lift an important negative signJugular venous pulse May be normal owing to a large, thin-walled right atrium, which can absorb the volume and pressure transmitted from the right ventricle through an incompetent tricuspid valve Large a and v waves late in the course of the disease, with development of right heart failureArterial pulses Usually normal Diminished volume late in the course of the disease due to severe right heart failure and decreased left ventricular stroke volumeHeart sounds First heart sound is widely split with loud tricuspid component secondary to delayed closure of the elongated anterior tricuspid leaflet, which has an increased excursion. Mitral component may be soft or absent in the presence of prolonged PR interval. Second heart sound usually is normal but may be widely split when the pulmonary component is delayed due to right bundle-branch block (RBBB).Additional heart sounds and murmurs Third and fourth heart sounds are commonly present, even in the absence of congestive heart failure (CHF). Summation of third and fourth heart sounds, especially with prolonged PR interval, can mimic an early diastolic murmur. The holosystolic murmur of tricuspid regurgitation is heard maximally at the lower left parasternal area and sometimes at the apex owing to the displaced location of the tricuspid valve; murmur intensity and duration increase during inspiration.

*HistoryCyanosis may be present at or shortly after birth but usuallydevelops within the first few years of life. First presentation may include poor feeding, cyanosis during feeding, fussiness, tachypnea, and agitation. Symptoms generally progress secondary to hypertrophy of the infundibular septum.Worsening of the RVOT obstruction leads to right ventricular hypertrophy, increased right-to-left shunting, and systemic hypoxemia. Cyanosisgenerally progresses with age and outgrowth of pulmonary vasculatureand demands surgical repair. Dyspnea on exertion is common. Squatting (a compensatory mechanism) is uniquely characteristic of a right-to-left shunt that presents in the exercising child. Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt and increases pulmonary blood flow. Hypoxic "tet" spells are potentially lethal, unpredictable episodes that occur even in noncyanotic patients with TOF. The mechanism is thought to include spasm of the infundibular septum, which acutely worsens the RVOT obstruction.These spells can be aborted with relatively simple procedures. The rare patient may remain marginally and imperceptibly cyanotic, or acyanotic and asymptomatic, into adult life. Severe cyanosis may present at birth in a patient with TOF and associated pulmonary atresia. Birth weight is low. Growth is retarded. Development and puberty may be delayed.PhysicalRight ventricular predominance on palpation May have a bulging left hemithorax Systolic thrill at the lower left sternal border Aortic ejection click Single S 2 - Pulmonic valve closure not heard Systolic ejection murmur - Varies in intensity inversely with the degree of RVOT obstruction More cyanotic patients have greater obstruction and a softer murmur. An acyanotic patient with TOF (pink tet) has a long, loud, systolic murmur with a thrill along the RVOT.Cyanosis and clubbing - Variable Squatting position Scoliosis - Common Retinal engorgement Hemoptysis

**It is preferably taken PA.Child is in upright position. The exposure time currently is less than 100 m seconds, reducing motion blurring. It is ideally taken during inspiration and the RIGHT diaphragm must be at the level of 9th to 10th rib. (ADULTS: 10th post. rib)CARINA: T5.Six complete anterior ribs (and ten posterior ribs) are clearly visible.Assessment of inspiratory adequacy is a simple process.It is ascertained by counting either the number of visible anterior or posterior ribs.If six complete anterior or ten posterior ribs are visible then the patient has taken an adequate inspiratory effort.Conversely, fewer than six anterior ribs implies a poor inspiratory effort and more than six anterior ribs implies hyper-expanded lungs.

ANTERIOR ribs are more OBLIQUEPOSTERIOR ribs are TRANSVERSE, and the ICS are NARROWER

*Costophrenic angle is sharp. Blunting means fluid in the pleural cavity (hydrothorax)250 cc is needed to obliterate costophrenic angle.

Cardiophrenic angle is sharp. Blunting means fluid in the pericardial cavity (pericardial effusion)

The trachea is placed usually just to the right of the midline. It is not deviatedThe right wall of the trachea should be clearly seen as the so-called right para-tracheal stripe (ARROW). The para-tracheal stripe is visible by virtue of the silhouette sign: air within the tracheal lumen and adjacent right lung apex outline the softtissue-density tracheal wall.Loss or thickening of the para-tracheal stripe intimates adjacent pathology.

* The cardiothoracic ratio should be 0.65 in CHILDREN A cardiothoracic ratio of greater than 0.65 (in a good quality film) suggests cardiomegaly.The highest point of the right diaphragm is usually 11.5 cm higher than that of the left.The right hemidiaphragm is higher than the left.LEFT IMAGE:There should be a decrease in density from superior to inferior in the posterior mediastinum.The retrosternal airspace should be of the same density as the retrocardiac airspace

The right hemidiaphragm is usually higher than the left. The outline of the right can be seen extending from the posterior to anterior chest wall.The outline of the left hemidiaphragm stops at the posterior heart border.Air in the gastric fundus is seen below the left hemidiaphragm.

*SLIGHLTY ENLARGEDAll pulmonary vessels are small, with hyperluscent lungs. A decrease in pulmonary vascularity is seen routinely, primarily due to a large right to left shunt across the VSD.

The boot-shaped heart sign is seen on the frontal chest radiograph of children with decreased pulmonary vasculature.The left cardiac border resembles the shape of a wooden boot.The toe of the boot is formed by the upward pointing cardiac apex, which makes an acute angle with the diaphragm.The upturned cardiac apex is ascribed to right ventricular hypertrophy and occurs in 65% of patients with TOF.***The narrower upper part of the boot results from a small or absent main pulmonary artery partly caused by a narrow infundibulum and hypoplastic main pulmonary artery. (BAT UNG ATIN ANG KAPAL)*Retrosternal space: ****Steph, the degree of septal defect, outflow obstruction, and stenosis determines the amount of mixed blood that will be circulated in the body. A greater amount of mixing between oxygenated and less oxygenated blood that is pumped into the body will in turn determine the degree of severity of symptoms as stated above. *Cyanosis may be present at or shortly after birth but usuallydevelops within the first few years of life.First presentation may include poor feeding, cyanosis during feeding, fussiness, tachypnea, and agitation.Symptoms generally progress secondary to hypertrophy of the infundibular septum.Worsening of the RVOT obstruction leads to right ventricular hypertrophy, increased right-to-left shunting, and systemic hypoxemia.Cyanosisgenerally progresses with age and outgrowth of pulmonary vasculatureand demands surgical repair.Dyspnea on exertion is common.Squatting (a compensatory mechanism) is uniquely characteristic of a right-to-left shunt that presents in the exercising child. Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt and increases pulmonary blood flow.Hypoxic "tet" spells are potentially lethal, unpredictable episodes that occur even in noncyanotic patients with TOF. The mechanism is thought to include spasm of the infundibular septum, which acutely worsens the RVOT obstruction.These spells can be aborted with relatively simple procedures.The rare patient may remain marginally and imperceptibly cyanotic, or acyanotic and asymptomatic, into adult life.Severe cyanosis may present at birth in a patient with TOF and associated pulmonary atresia.Birth weight is low.Growth is retarded.Development and puberty may be delayed.

*REFER TO PICTURE WHILE EXPLAININGNormally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped through the aorta out to the body.In tetralogy of Fallot, blood flow within the heart varies, and is largely dependent on the size of the ventricular septal defect, and how severe the obstruction in the right ventricle is.With mild right ventricle obstruction, the pressure in the right ventricle can be slightly higher than the left. Some of the oxygen-poor (blue) blood in the right ventricle will pass through the VSD to the left ventricle, mix with the oxygen-rich (red) blood there, and then flow into the aorta. The rest of the oxygen-poor (blue) blood will go its normal route to the lungs. These children may have slightly lower oxygen levels than usual, but may not appear blue. With more serious obstruction in the right ventricle, it is harder for oxygen-poor (blue) blood to flow into the pulmonary artery, so more of it passes through the VSD into the left ventricle, mixing with oxygen-rich (red) blood, and then moving on out to the body. These children will have lower than normal oxygen levels in the bloodstream, and may appear blue, especially whenever the pressure in the right ventricle is very high and large amounts of oxygen-poor (blue) blood passes through the VSD to the left side of the heart.

***Echocardiogram in a patient with the tetralogy of Fallot. This parasternal long-axis two-dimensional view demonstrates anterior displacement of the outflow ventricular septum that resulted in stenosis of the subpulmonic right ventricular outflow tract, overriding of the aorta, and an associated ventricular septal defect. Ao, overriding aorta; LA, left atrium; LV, left ventricle; RV, right ventricle *****Magnetic resonance image of the heart in an infant with tetralogy of Fallot. This image shows a large ventricular septal defect and right ventricular hypertrophy. Note the descending aorta is on the right, consistent with a right-sided aortic arch.

*Interventricular septum*Aorta is still delineated from the RVAorta is over the LV**Magnetic resonance image of tetralogy of Fallot in an infant (same patient as in Image 4). This image shows a large ascending aorta and the presence of pulmonary atresia. The oval shape of the descending aorta is secondary to large collateral vessels.

**Angiogram in an infant with tetralogy of Fallot and a right-sided aortic arch. This image shows the pulmonary artery is hypoplastic, and the branch pulmonary arteries have a characteristic seagull appearance.

**Angiogram in an infant with tetralogy of Fallot and a right-sided aortic arch. This image shows the pulmonary artery is hypoplastic, and the branch pulmonary arteries have a characteristic seagull appearance. ********

interactive case 4 cardiac b

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