dgpk guideline
TRANSCRIPT
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correspondence to: Harald Bertram, MD Pediatric Cardiology and Intensive Care, Hannover Medical School
DGPK guideline
no conflicts of interest no disclosures
Pulmonary Atresia with Intact Ventricular Septum
(PA-IVS) • H. Bertram, Medizinische Hochschule, Hannover • M. Hofbeck, Universitätsklinik,Tübingen • A. Horke, Medizinische Hochschule, Hannover & DGPK guideline committee
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Guideline PA-IVS Definition
- 75-80% isolated membraneous atresia of the pulmonary valve; combined with infundibular atresia in 18-25% - intact ventricular septum - hypoplasia of tricuspid valve, right ventricle, and pulmonary artery (PA) trunk - significant hypertrophy of the right ventricle - usually normal sized branch PAs - right atrial enlargement - atrial septal defect
• malformation of right-sided cardiac structures with heterogeneous morphological appearance
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Guideline PA-IVS anatomic variations
RA LA
LV
RV
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Piers E.F Daubeney et al. Pulmonary atresia with intact ventricular septum : Range of morphology in a population-based study Journal of the American College of Cardiology, Volume 39, Issue 10, 2002, 1670 - 1679
Guideline PA-IVS anatomic variations
RV size and morphology
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Guideline PA-IVS anatomic variations
Tricuspid valve malformation
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Guideline PA-IVS basic information
• concomitant Ebstein's malformation in 8-9 %
• in rare cases (~ 5 %) with severe tricuspid regurgitation, RV and tricuspid valve annulus are dilated, with poor systolic RV function
• prevalence PA-IVS: 0,3 – 0,45 / 10.000 live births PAN: 0.3 % of all congenital heart defects
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• considerably variable anomalies of the coronary arteries: => stenoses / sinusoids / fistulas
Guideline PA-IVS Definition – Basic information
- clear correlation to the grade of RV hypoplasia - myocardial sinusoids or small fistula between RV cavity and coronary arteries are frequent: 40-50%
RV-angiogram (lateral view) displaying multiple small connections to both coronary arteries
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=> RV-dependent coronary circulation (RVDCC) (7-9%) • coronary ectasia or very large fistulas • coronary fistulas without aorto-coronary connection • stenosis or discontinuations of large coronary artery branches • coronary perfusion of relevant portions of the LV being supplied by the RV
Piers E.F Daubeney et al. Pulmonary atresia with intact ventricular septum : Range of morphology in a population-based study Journal of the American College of Cardiology, Volume 39, Issue 10, 2002, 1670 - 1679
Guideline PA-IVS Definition – Basic information
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RV-dependent coronary circulation • coronary perfusion of relevant portions of the LV being supplied by the RV
Guideline PA-IVS Diagnostics
must be excluded before valvotomy of
the pulmonary atresia is considered RV angiogramm (RAO) with atretic RVOT and
filling of major part of LCX and LAD
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Hemodynamics • duct-dependent pulmonary blood flow • inadvertent interatrial communication with right-to-left shunt • suprasystemic RV pressure • tricuspid regurgitation (variable)
Guideline PA-IVS basic information
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• differential diagnoses: - tricuspid atresia, critical pulmonary stenosis, Ebstein’s anomaly, severe tricuspid regurgitation, pulmonary atresia with VSD - "functional pulmonary atresia" (in pulmonary hypertension and / or failing right ventricle)
Guideline PA-IVS basic information
clinical presentation: • neonates/infants: - mild cyanosis due to right-to-left shunt at atrial level - duct closure causes acute severe cyanosis - severe cyanosis combined with congestive heart failure in the rare case of restrictive PFO - in the rare case of severe enlargement of RA due to severe TR respiratory distress due to bronchial compression • older children: duct dependent / shunt dependent cyanotic heart disease
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Guideline PA-IVS Diagnostics
Goal: displaying cardiac and coronary anatomy with respect to the therapeutic goal of neonatal RV decompression • membranous vs. infundibular atresia • extent of RV hypoplasia and hypertrophy - absence of trabecular part and apex? - the size of the tricuspid valve (TV) correlates well with RV size: → tricuspid valve annulus diameter (TVA) (apical and subcostal 4-chamber view in end-diastole) including Z-score calculation; relation to mitral valve annulus diameter → degree of TR; calculation of RV pressure • size of pulmonary valve annulus, pulmonary artery trunk and proximal branch arteries • size and morphology of the arterial duct (potential ductal stenting!) and atrial septal defect (restriction?) • extent of coronary anomalies: - myocardial sinusoids / small coronary fistulas - potential right ventricule dependent coronary circulation?
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Guideline PA-IVS Diagnostics
• the size of the tricuspid valve (TV) correlates well with RV size: → tricuspid valve annulus diameter (TVA) (apical and subcostal 4-chamber view in end-diastole) including Z-score calculation; relation to mitral valve annulus diameter
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Guideline PA-IVS Diagnostics
→ for complete imaging, both echocardiography and additional catheterization are usually required
→ for practical purpose, diagnostic catheterization and first catheter palliation will be performed during one intervention in most centers
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morphological classification
(echocardiography / angiography)
sufficient RV-size • TV Z-score* > -2,5
• membraneous atresia with well developed infundibulum
• variable TR • no major coronary sinusoids
Intermediate forms • ‚borderline‘-RV, often hypoplastic
trabecular RV-component (‚bipartite‘) • TV Z-score* -2,5 bis -5
• patent infundibulum +/- subvalvular stenosis
+/- hypoplastic PV-annulus • variable TR
• +/- small sinusoids
severe RV-hypoplasia • TV Z-score* < -5 • no trabecular RV
• infundubulum subatretic / atretic • mostly no TR
• often large sinusoids • +/- RV-pressure-dependent
coronary perfusion
group A group B group C
Guideline PA-IVS Diagnostics
* TV-annulus > 8 mm * TV-annulus > 5 mm und < 8 mm
* TV-annulus < 5 mm
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Ao
PA
morphological classification
(echocardiography / angiography)
initial therapy
sufficient RV-size • TV Z-score* > -2,5
• membraneous atresia with well developed infundibulum
• variable TR • no major coronary sinusoids
Intermediate forms • ‚borderline‘-RV, often hypoplastic
trabecular RV-component (‚bipartite‘) • TV Z-score* -2,5 bis -5
• patent infundibulum +/- subvalvular stenosis
+/- hypoplastic PV-annulus • variable TR
• +/- small sinusoids
severe RV-hypoplasia • TV Z-score* < -5 • no trabecular RV
• infundubulum subatretic / atretic • mostly no TR
• often large sinusoids • +/- RV-pressure-dependent
coronary perfusion
RF-perforation or surgical
commissurotomy
RF-perforation + ductal stenting or
surgical valvotomy +/- RVOT-enlargement +/- AP-shunt +/- Atrioseptostomy
ductal stent + BAS or
AP-shunt +/- BAS
group A group B group C
Guideline PA-IVS Therapy
• Neonatal RV decompression (interventional or surgical valvotomy) => prerequisite for subsequent RV growth → antegrade pulmonary blood flow → decreasing RV pressure → reducing pre-existing TR
• Additional pulmonary perfusion through the duct is still needed in some patients to allow for adaptation of the RV → low-dose PG infusion might be continued
Goal (group A & B): biventricular repair with separation of systemic and pulmonary circulation
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Guideline PA-IVS case I
1st day of life, 2.4 kg
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Guideline PA-IVS case I
1st day of life, 2.4 kg
9 mm
5 mm
Moderate TR Z-score – 2.1
good sized main PA
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Guideline PA-IVS case I
3rd day of life, 2.3 kg: catheter valvotomy
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Guideline PA-IVS case I
3rd day of life, 2.3 kg: catheter valvotomy
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Guideline PA-IVS case I
3rd day of life, 2.3 kg: catheter valvotomy
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Guideline PA-IVS case I
3rd day of life, 2.3 kg: catheter valvotomy
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TV-Ring 12 mm Z-score -1.3
Guideline PA-IVS case I
TTE nach 6 Mo
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Guideline PA-IVS case II
• HF-perforation and balloon dilation (3 & 8 mm balloons)
• PG E continued for 5 days
• rapid regression of TR after successful catheter valvotomy
• no further intervention during f/u (8.5 y)
PA-IVS; 4 kg; PG E – infusion; severe TR (IV°); subsequent massive RA-dilatation
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Guideline PA-IVS case III
PA-IVS; 2.8 kg; ‚good sized‘ RV, TV Z-score -1.1; moderate TR;
=> RF perforation + balloon; PG E continued
ductal stenosis despite PG E, decreasing SO2
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LV LA
Guideline PA-IVS case III
antegrade ductal stenting with coronary stent
rationale for ductal stenting: 40 % of pts after interventionally perforated atresia and nearly 50 % of pts after surgical valvotomy need a second intervention to secure sufficient pulmonary blood flow before hospital discharge
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Guideline PA-IVS Follow-up / case III
4.5 y after valvotomy; TV Z-score: 0.4
2 y after valvotomy: mild PR, stented duct with residual flow
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Guideline PA-IVS case IV
PA-IVS; 4.3 kg; severe RV-hypertrophy; good sized TV annulus; good sized PV annulus
RF perforation + balloon
ductal stenting (2 stents)
persistent muscular RVOTO
redilation RVOT (balloon)
surgical RVOT augmentation
no further intervention for the next 6 y; normal exercise capacity
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initial therapy
reintervention
HF-perforation or surgical
commissurotomy
HF-perforation + ductal stenting or
surgical valvotomy +/- RVOT-enlargement +/-
AP-shunt +/- atrioseptostomy
ductal stent + BAS or
AP-shunt +/- BAS
rare ! • RVOT redilatation • RVOT-augmentation (fixed subvalvular stenosis) • TV-reconstruction (severe TR)
surgical completion of univentricular
circulation • bidirectional CPA • modified Fontan
procedure
sufficient RV-growth • no cyanosis => no reintervention • cyanosis => interventional ASD/PFO closure
insufficient RV-growth • CPA (1½ ventricle) • w/o additional RVOT augment. (subvalv.stenosis / small PV-annulus) • TV-reconstruction (severe TR)
group A: sufficient RV-size group B: Intermediate forms group C: severe RV-hypoplasia
• Reinterventions during follow-up focus on → residual RVOT stenosis → surgical treatment of severe TR → ASD closure to achieve separation of systemic and pulmonary circulation → pulmonary valve replacement [in the long-term]
• Insufficient RV growth => ‘1½-ventricle repair’ → surgical removal of residual RVOT obstruction combined with a → bidirectional cavo-pulmonary anastomosis → closure of the ASD
• severe RV hypoplasia => univentricular palliation according to the ‘Fontan principle’
Guideline PA-IVS Therapy
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• life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory
Guideline PA-IVS Follow-up
• besides tricuspid valve regurgitation and pulmonary valve regurgitation, regular follow-up visits should assess growth and function of RV and TV (Z-scores, identical reference group!)
• objective examinations of exercise capacity and Holter ECGs every 2-3 years (school age)
• residual findings, which might require further diagnostic imaging (MRI, re-cath) and potential treatment: - RVOT reobstruction - tricuspid regurgitation
- high-grade pulmonary regurgitation - ASD
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Guideline PA-IVS Follow-up / Reinterventions
Chubb et al. JACC 2012
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Guideline PA-IVS Prognosis
• Perioperative morbidity and mortality is considerable in infancy (highest in patients with low birth weight, concomitant Ebstein's anomaly, dilated thin-walled RV, and RVDCC)
• Positive prognostic factors to achieve biventricular repair with separation of systemic and pulmonary circulation (~ 50 % of cases) are: - larger initial TV and PV annulus diameter - a ratio of TVA / MVA of > 0.5 - tcSO2 > 90 % at the age of one year
• Despite absolute growth of TV and RV, a real catch-up growth (increase in tricuspid valve Z-score of > 2 points) is observed in only 10-15 % of cases
• Exercise capacity will be reduced in most patients even after biventricular repair due to the ongoing restrictive RV physiology with diastolic RV-dysfunction
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Dealing with Z-scores / Z-values Guideline PA-IVS
Z = [measured value - mean of the population] / standard deviation of the normal population • The Z-value quantifies the position of an individually measured size within a sample population; the measurement unit is the standard deviation above (+ ) or below ( -) the population´s mean value • The validity of the values is limited to - a comparable normal population with normal distribution of the data - the same measurement method for the definition of the mean and standard deviation
• To be used in the clinical setting, the sample size should have been large enough (especially in young infants!). • Mean and standard deviation may vary between the various investigators or with body size • For observational studies the same reference data and the same method of measurement should be used throughout
Chubb et al. Annals of Pediatric Cardiology 2012
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Daubeney et al. 1999
Hanley et al. 1993
Guideline PA-IVS
Rohdaten: Rowlatt et al. Postmortem !
Dealing with Z-scores / Z-values
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Guideline PA-IVS Dealing with Z-scores / Z-values
Pettersen et al. Journal of the American Society of Echocardiography, Volume 21, Issue 8, 2008, 922 - 934
Zilberman et al. Ped Cardiol 2005
782 pts; 125 pts < 1 y
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reference 3mm 4mm 5 mm 6mm 7mm 8mm 9mm 10mm 11mm 12mm 13mm 14 mm Detroit data, Pettersen graphik -3 -2,5 -1,3 -0,3 0,5 J Am Soc Echocard parameterz -7,59 -6,07 -4,98 -3,93 -3,12 -2,42 -1,8 -1,24 -0,74 -0,28 0,14 0,53 Cincinatti data, Zilberman nomogramm boys -2,4 -1,16 -0,1 Ped Cardiol 2005 nomogramm girls -2,15 -0,99 0 parameterz boys -4,56 -3,58 -2,75 -2,03 -1,4 -0,84 -0,32 0,15 0,58 0,97 parameterz girls -4,59 -3,61 -2,78 -2,06 -1,43 -0,86 -0,35 0,12 0,55 0,95 Wessex data nomogramm -6,3 -3,5 -1,2 0,85 Daubeney 1999 parameterz -11,9
4 -9,7 -7,8 -6,15 -4,7 -3,41 -2,23 -1,16 -0,18 0,74 Rowlatt-Daten (Patho) nomogramm -5,3 -4,6 -4 -3,3 -2,7 -2,1 -1,4 -0,8 -0,2 0,5 1,1 1,8
in a normal sized newborn (0,2 m2), a measured TV-diameter of X mm corresponds to a TV Z-score of
Guideline PA-IVS Dealing with Z-scores / Z-values
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Guideline PA-IVS Dealing with Z-scores / Z-values
Implications for the PA-IVS guideline: • most of the (older) surgical publications use reference data of inadequatly small autopsy series, which should no longer be applied today • the best validated reference data for the calculation of TV Z-scores are from Pettersen et al. [,Detroit data'], which are available for online calculation under www.parameterz.com
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Guideline PA-IVS Dealing with Z-scores / Z-values
modified from Chubb et al. JACC 2012, using Z-scores from Pettersen 2008
- 2
- 4