Reconstruction of pulmonary pathway in congenital cardiac anomalies Pr Abid Abdelfatteh 5th Mauritanian cardiology meeting 31st October – 1st November 2015 Nouakchott Mauritania

Pulmonary pathway in congenital Cardiac anomalies Normal Abnormal RV-PA discontinuity Abnormal flow Obstruction Regurgitation RV-PA continuity Normal flow Interruption Stenosis Ex Aortic stenosis T4F

Abnormal Pulmonary Pathway Native congenital Repaired congenital malformations malformations T4F pulmonary atresia Truncus arteriosus.

Abnormal Pulmonary Pathway Diagnosis -Clinical symptoms * Dyspnea * Cyanosis * Rigt heart failure Chestxray EKG Echocardiography +++ Angioscanner Angio MRI +++ Cardiac catheterisation +++

Reconstruction in obstructive pulmonary pathway Interruption - Common arterial trunk Reconstructive surgery direct interposition of VD-PA conduit - Pulmonary atresia Catheter perforation Surgical restoration of pulmonary pathway Patch or conduit

Reconstruction of pulmonary pathway In pulmonary stenosis Infundibular stenosis Surgical resection of fibrous tissue and muscle Patch closure

Reconstruction of pulmonary pathway Valvar stenosis - With normal pulmonary annulus * Percutaneous catheter commissurotomy * Surgical commissurotomy - With small pulmonary annulus Surgical enlargement with transannular patch

Reconstruction of pulmonary pathway In pulmonary trunk stenosis Surgical patch enlargement Without cusp With cusp RV – PA Conduit

Reconstruction of pulmonary pathway In distal pulmonary stenosis Surgical patch Catheter dilatation and stenting

Reconstruction of pulmonary pathway Multifocal stenosis Extended enlargement

Reconstruction of pulmonary pathway Hybrid procedure

Reconstruction of pulmonary pathway In abnormal coronary arteries Myomectomy VD –PA conduit

Rare Abnormal Pulmonary Arteries

Pulmonary Regurgitation Agenesis of the pulmonary valve Post repair of congenital cardiac malformations   - Diagnosis * clinical symptoms * EKG - Holter * echocardiography * cardiac cath .morphological informations * MRI .functional informations Degree of pul and tricuspid regurgitation RV function and dilatation

Angio MRI Pulmonary regurgitation RV dilatation RV dysfonction MR + 3D reconstruction Araldi Pulmonary regurgitation RV dilatation RV dysfonction Tricuspid regurgitation 15

Pulmonary insufficiency Seen every time pulmonary valve was involved in initial surgical management : commissurotomy transannular patch RV-PA conduit

Why do Tissue Valves Fail? Most common valve failure mode is structural valve deterioration (SVD) Calcification remains the primary cause of SVD

…………….severe pulmonary regurgitation alone, requiring valve insertion, is uncommon…….. World Congress of Paediatric Cardiology 1989

Natural History of PR One of the reason for the lack of appreciation of the impact of PR is its very long preclinical natural history At age 20 years, only 6% of the pt had symptoms, but the incidence increased to 29% at age 40 years Shimazaki Y Thorac Cardiovasc Surg1984;32:257-9

Natural History of PR At the time of ToF repair the RV is hypertrophied and its compliance is low; the diameters of the central PA are either hypoplastic or low-normal, and their capacitance is low. The heart rate is relatively high, which leads to a relatively short duration of diastole The combination of these factors limits the degree of pulmonary regurgitation.

Natural History of PR Over time the increase in RV stroke volume leads to progressive rise in the size and compliance of the central PA and to increased RV compliance Combined with a longer duration of diastole as HR decreases with age, these changes lead to progressive increase in the degree of PR

The number of pts free of reinterventions for PVR decrease during the 3rd-4th decade

During the past 2 decades it has become apparent that PR is a key driver of RV failure but the Timing for PVR remains Controversial

Criteria for PV Replacement Exercise intollerance Pt with symptoms Exercise intollerance Heart failure sVT syncope PVR surgically or transcatheter

Criteria for PV Replacement Pt asymptomatic with PR ≥ 25-35% + at least 2 criteria RV EDVi ≥ 150 mL/m2 or RV/LV >1.5 RV ESVi ≥ 80 mL/m2 RV volumes and function RV EF ≤ 45% ≤ 65% of the predicted VO2 max QRS ≥ 180 msec (better before 180 because no improvments after PVR) TR ++ Residual VSD RVOTO (RVP 2/3 LVP or ΔP ≥ 50 mmHg AR ++ LV Dysfunction

Why timing is so important? Why timing is so difficult? What do we know OR DON’T know? Which are possible future directions?

The Timing! Certainly PVR should be performed when patients develop first symptoms as dyspnea, but it is not infrequent that they may have advanced RV dysfunction by the time complain of symptoms Serial exercise testing may help to delineate subtle changes in exercise capacity before the pt becomes symptomatic.

Pulmonary insufficiency Minimal or moderate: follow up medical treatment Severe : Pulmonary valvulation Surgical management RV-AP conduit pulmonary valve replacement Percutanous management : percutanous pulmonary valve

Surgical treatment of pulmonary regurgitation Pulmonary valvulation Pulmonary bioprosthesis RV-PA conduit with valve substitute homograft contegra conduit conduit with bioprosthesis Redo surgery ++++

Percutaneous treatment of pulmonary regurgitation Percutaneous valved conduit insertion

Melody® Transcatheter Pulmonary Valve 18mm Contegra modified-bovine jugular vein with valve segment NuMed Platinum Iridium Stent 28 mm length Crimped down to 6mm, re-expanded 18mm up to 22mm Balloon Expandable system SM: Added poin 2 – valves can be self expandable or self exp – so it’s important to underline it. 32

Melody

Melody® Indications Extend the functional life of the RV-PA conduit Restore & maintain pulmonary valve competence Relieve conduit stenosis without inducing regurgitation

Complications/Failures ► Homograft rupture ► Coronary compression ► Melody dislodgement ► Pulmonary artery perforation ► Tricuspid damage ► Death (coronary compression)

Limitations for Melody RVOT surgical reconstruction without conduit (?) RVOT larger than 22 mm Unfavourable anatomy for secure stent anchorage

Immediate results Worlwide experience ≅ 2500 patients Successful implants 94 % Complications/Failures 6%

Conclusion I In congenital cardiac malformations, pulmonary pathway can be abnormal : obstruction or regurgitation Technical reconstruction is to be adapted to the type of malformation In reconstruction of pulmonay pathway it is crucial to relieve as much as possible pulmonary obstruction and to preserve pulmonary valve competence

Conclusion II After repair of cardiac malformations with abnormal pulmonary pathway regular follow up is mandatory - To detect residual or recurrent pulmonary stenosis - To detect and evaluate pulmonary regurgitation  appropriate type and time of management : In pulmonary stenosis : surgery when RV-PA gradient ≥ 50 mmHg In pulmonary regurgitation : pulmonary valvulation before severe deterioration of RV function ( surgery or percutanous treatment) to preserve the quality of the RV .

Thank you