a review of congenital heart disease february 11, 2008
TRANSCRIPT
![Page 1: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/1.jpg)
A Review ofCongenital Heart Disease
February 11, 2008
![Page 2: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/2.jpg)
Presentation
• Symptomatic infants with congenital heart disease will have one or more of the following three main presentations:– Cyanosis– Heart Failure– Shock
• Infants with ductal dependent lesions usually present in the first week of life and are often critically ill
![Page 3: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/3.jpg)
Presentation
• Infants with heart failure have nonspecific symptoms and signs, which are frequently very different from those of older children and adults.
• Symptoms can include poor feeding, weight loss, FTT, palpitations, apnea, chest pain, syncope, and fever.
• Signs can include murmurs, hypertension, dysrhythmias, respiratory distress or failure, cyanosis, heart failure, and shock.
![Page 4: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/4.jpg)
Presentation: Vitals
• Hypertension– Can be a manifestation of underlying cardiovascular
diseases including coarctation and aortic stenosis
• Hypotension– Can be due to poor cardiac output from sepsis or shock
• A difference in blood pressure and/or pulses between the extremeties suggests coarctation of the aorta or an interruption of the aortic arch
![Page 5: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/5.jpg)
Presentation
• Although a significant murmur implies the presence of cardiac disease, the absence of a murmur does not rule out underlying heart disease
• Some heart defects (for example transposition of the great arteries) may not have a murmur initially or may have only a nondescript murmur (e.g. single ventricle)
![Page 6: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/6.jpg)
![Page 7: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/7.jpg)
Physical Exam
• Abnormal heart sounds are a clue that underlying heart disease is present
• A wide, fixed, split S2 is characteristic of an atrial septal defect
• With pulmonary atresia or truncus arteriosus, S2 is single
![Page 8: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/8.jpg)
Physical Exam
• After the first day of life, a systolic ejection click is always abnormal and is found with dilation of the aorta or pulmonic trunk
• The murmur of a patent ductus arteriosus is a continuous, even murmur “machine-like,” best heard in the left scapular area
![Page 9: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/9.jpg)
Presentation
• Hepatosplenomegaly can occur with right-sided heart failure
• Palpation of the pulses can reveal bounding pulses with a wide pulse pressure indicative of a patent ductus arteriosus or aortic insufficiency, or the weak thready pulses occurring with low cardiac output
• Ashen, blue, grey, cold, clubbed, edematous and clammy extremities suggest a cyanotic lesion
![Page 10: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/10.jpg)
Presentation
• Because specific cardiac defects are associated with chromosomal abnormalities, abnormal facies, or skin lesions can be a clue to underlying congenital heart disease
![Page 11: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/11.jpg)
Heart Failure
• The usual age at which a large left-to-right shunt will become symptomatic is about six weeks
![Page 12: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/12.jpg)
Heart Failure
• At birth there is a marked initial decrease in the pulmonary vascular resistance followed by a continued slow drop over the next 2-6 weeks of life, with more blood being shunted from left to right
• Infants with these defects are asymptomatic at birth, and symptoms appear and gradually worsen over the next few weeks
![Page 13: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/13.jpg)
Heart Failure
• The classic heart failure triad in infancy is tachypnea, tachycardia, and hepatomegaly
• Pallor and diaphoresis are additional findings in infants
• Rales may be heard on auscultation, but the absence of rales does not rule out heart failure
![Page 14: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/14.jpg)
Ductal-Dependent Congenital Heart Defects
• Many patients with congenital heart defects depend on a patent ductus arteriosus for blood flow
![Page 15: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/15.jpg)
PDA
• When the ductus arteriosus closes, patients with ductal-dependent lesions develop symptoms of acute heart failure and cardiogenic shock with circulatory collapse
• Closure of the ductus usually occurs during the first week of life
![Page 16: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/16.jpg)
PDA
• Functional closure occurs in the first 10-14 hours of life, although anatomic closure can be delayed for 2-3 weeks because of hypoxia, acidosis, and prematurity
• The classic presentation of patients with ductal-dependent lesions is an infant, asymptomatic at birth and in the first few hours or days of life, who develops shock/circulatory collapse toward the end of the first week of life or in the first few weeks of life
![Page 17: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/17.jpg)
Who gets Prostin?
• For symptomatic patients with ductal-dependent congenital heart defects, a PGE1 infusion will keep the ductus arteriosus open until definitive therapy can be undertaken via interventional cardiac catheterization or surgery
![Page 18: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/18.jpg)
Prostin
• PGE1 is given as an infusion, using the mimimum effective dose because side effects are dose dependent and include:– Apnea– Hypotension– Bradycardia– Seizures– Tremors
• Because of the side effects, patients are usually intubated prior to beginning PGE1
![Page 19: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/19.jpg)
PDA in preemies• PDA is the most common cardiovascular abnormality in
preterm infants
• It occurs in up to 60% of infants born at less than 28 weeks gestation
• A significant L R shunt through a PDA increases the morbidity and mortality of these infants.
• Cyclooxygenase inhibitors such as indomethacin or ibuprofen are used to induce closure of the PDA in preterm infants
![Page 20: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/20.jpg)
Cyanotic Infants
• The “five Ts” are common etiologies of cyanotic congenital heart disease:– Tetralogy of Fallot– Transposition of the great arteries– Truncus arteriosus– Tricuspid valve abnormalities– Total anomalous pulmonary venous return
• The letter “S” is added to include severe or “critical” pulmonic stenosis
![Page 21: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/21.jpg)
Cyanotic lesions
• The first priority is to determine whether the etiology of the cyanosis is cardiac or noncardiac
• The hyperoxia test or oxygen challenge test can make this clear.
• The patient is placed on 100% oxygen:– If pulmonary disease is present, the PaO2 should rise
by 30 mmHg or to >150 mmHg, and the pulse ox should rise by at least 10%
– There will be negligible or minimal improvement if the cyanosis is due to CHD
![Page 22: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/22.jpg)
Cyanotic CHD with Decreased Pulmonary Blood Flow
• Cyanotic CHD with decreased pulmonary blood flow include:– Severe tetralogy of Fallot– Pulmonary atresia – Tricuspid atresia– Severe Ebstein anomaly– HRHS– Critical or severe pulmonic stenosis
![Page 23: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/23.jpg)
Cyanotic CHD with Decreased Pulmonary Blood Flow
• These lesions usually are ductal dependent
• These patients will present critically ill and cyanotic (and sometimes with heart failure and shock) in the first few hours or days of life when the ductus arteriosus closes
• The management of such infants involves supporting the ABCs and administering PGE1
![Page 24: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/24.jpg)
Tetralogy of Fallot
• Tetralogy of Fallot is the most common cyanotic congenital heart defect after infancy, accounting for 5%-10% of all congenital heart disease
• It consists of:– VSD– Obstruction of the RV outflow tract– Overriding aorta– RVH
![Page 25: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/25.jpg)
Tetralogy of Fallot
![Page 26: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/26.jpg)
Tetralogy of Fallot
• Patients with TOF usually present as older infants with paroxysmal hypercyanotic attacks (tet spells)
• However, TOF can present in neonates, critically ill and severely hypoxic with extreme pulmonic stenosis
• These infants are dependent on a PDA for pulmonary blood flow, and prostin is necessary
![Page 27: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/27.jpg)
Tetralogy of Fallot
• Conversely are the “pink tets” who have mild right ventricular outflow tract obstruction and present with heart failure from the large LR shunt and have little or no cyanosis
• Older infants and children can present with a tet spell, cyanosis, murmur, exercise intolerance, dyspnea on exertion, clubbing, poor growth, or failure to thrive
![Page 28: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/28.jpg)
Tetralogy of Fallot
• Classic physical findings include:– Holosystolic VSD murmur at the left 3rd intercostal
space– A systolic diamond-shaped pulmonic stenosis
murmur at the left 2nd intercostal space– An abnormal second heart sound split with a soft
P2
![Page 29: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/29.jpg)
Tetralogy of Fallot
• Chest radiograph findings are:– cardiomegaly (especially RV)– Decreased pulmonary vascularity– The characteristic “boot-shaped” heart created by
a concavity in the left heart border usually occupied by the pulmonary artery
![Page 30: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/30.jpg)
“Boot Shaped”
![Page 31: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/31.jpg)
Tetralogy of Fallot
• “Boot-shaped”
![Page 32: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/32.jpg)
Tetralogy of Fallot
![Page 33: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/33.jpg)
Tetralogy of Fallot
• Tet spells occur especially during the first 2 years of life
• They can be precipitated by any physical activity or can occur spontaneously and can last a few minutes or for hours
• Since there is a fixed right ventricular outflow tract obstruction, increased right-to-left shunting occurs
![Page 34: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/34.jpg)
Tetralogy of Fallot
• Factors presdisposing to a tet spell include:– Dehydration– Anemia– Acidosis– decreased systemic vascular resistance– increased catecholamine levels
![Page 35: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/35.jpg)
Tetralogy of Fallot
• Treatment:– Calming the child– Supplemental oxygen– Morphine calms/sedates the child, depresses
respiration, decreases SVR, and decreases RVOT obstruction
– Volume infusion can increase the RV preload and correct systemic hypotension
– Propranolol is given as a negative inotropic on the RVOT
![Page 36: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/36.jpg)
Tricuspid Valve Abnormalities
• Tricuspid valve abnormalities include:– Tricuspid valve stenosis– Tricuspid valve displacement (Ebstein anomaly)– HRHS
![Page 37: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/37.jpg)
Ebstein Anomaly
• An Ebstein anomaly is the downward displacement of an abnormal TV into the RV
• May be due to maternal use of lithium
• Part of the TV—the anterior cusp—has some attachment to the valve ring, and the other valve leaflets are attached to part of the RV wall
![Page 38: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/38.jpg)
Ebstein Anomaly
![Page 39: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/39.jpg)
![Page 40: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/40.jpg)
Cyanotic Congenital Heart Diseasewith Increased Pulmonary Blood Flow
• Includes:– Transposition of the great arteries– Truncus arteriosus– Total anomalous pulmonary venous return
![Page 41: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/41.jpg)
Transposition of the Great Arteries
• Most frequent diagnosis in critically ill neonates with cardiac disease
• Pulmonary artery arises from the LV and the aorta from the RV
![Page 42: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/42.jpg)
Transposition of the Great Arteries
![Page 43: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/43.jpg)
Transposition of the Great Arteries
• Some mixing of blood is needed for survival through a:– Patent foramen ovale,– PDA, or– VSD
![Page 44: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/44.jpg)
Transposition of the Great Arteries
• “egg on a string” appearance– Narrow
mediastinum and small thymus
– Narrow cardiac silhouette
![Page 45: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/45.jpg)
Truncus Arteriosus
• A single arterial trunk overrides a VSD and receives mixed arterial and venous blood from the RV and LV
• This one arterial trunk provides blood flow to the systemic, pulmonary, and coronary circulations
• The one “truncal” valve is an abnormally formed semilunar valve
![Page 46: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/46.jpg)
Truncus Arteriosus
• First few hours and days of life:– Pulmonary vascular resistance is highpulmonary
blood flow is normal
• Later during first month of life:– Pulmonary blood flow increases with the
postnatal decrease in pulmonary vascular resistanceheart failure
![Page 47: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/47.jpg)
Truncus Arteriosus
• Cyanosis can be mild because of the increased pulmonary blood flow
• Eventually Eisenmenger syndrome occurs if untreated
![Page 48: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/48.jpg)
Truncus Arteriosus
![Page 49: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/49.jpg)
Truncus Arteriosus
![Page 50: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/50.jpg)
Total Anomalous Pulmonary Venous Return
• The pulmonary veins return to and enter a structure other than the left atrium
• Can be partial:– Some oxygenated blood enters the LA, and some
enters another anomalous structure– acyanotic
![Page 51: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/51.jpg)
Total Anomalous Pulmonary Venous Return
• Can be total:– None of the pulmonary veins enter the LA– Cyanotic: complete mixing of both systemic and
pulmonary venous return either at or before the RA
– The mixed blood in the RA either is ejected into the RV or through an ASD or PFO into the LA
![Page 52: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/52.jpg)
Total Anomalous Pulmonary Venous Return
• When the anomalous pulmonary veins enter the brachiocephalic vein and the persistent left superior vena cava, there is a “snowman” or “figure 8” appearance created by a large supracardiac shadow along with the normal cardiac shadow
![Page 53: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/53.jpg)
Total Anomalous Pulmonary Venous Return
• “Snowman”or “Figure 8”
![Page 54: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/54.jpg)
Eisenmenger Syndrome
• When a large LR shunt eventually causes increased pulmonary blood flow and volume overload to the lungs, resulting in pulmonary vascular disease/pulmonary hypertension
• This causes a reversal to a RL shunt and cyanosis, often by teenage years or as a young adult
![Page 55: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/55.jpg)
Eisenmenger Syndrome
• Symptoms:– Cyanosis– Exertional dyspnea– Fatigue– Hemoptysis– palpitations
![Page 56: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/56.jpg)
Eisenmenger Syndrome
• Physical exam reveals a loud P2 from pulmonary HTN
• The CXR will reveal decreased pulmonary vascularity
• EKG shows RVH
• Therapy involves pulmonary vasodilator therapy
![Page 57: A Review of Congenital Heart Disease February 11, 2008](https://reader035.vdocuments.mx/reader035/viewer/2022070521/58f059841a28ab7d438b45d9/html5/thumbnails/57.jpg)
References
• Mace, S. Broken Hearts: Infants With Acyanotic Congenital Heart Disease. CDEM. 2007; 21(11): 2-9.
• Mace, S. Broken Hearts: Infants With Cyanotic Congenital Heart Disease. CDEM. 2007; 21(11): 12-23.
• Multimedia Library. Childrens Hospital Boston. http://www.childrenshospital.org/
• Beerman, L; et al. Cardiology. Atlas of Pediatric Physical Diagnosis. Fifth Edition. 2007; 127-160.