Pulmonary Atresia and Intact Ventricular Septum

A Collaborative approach to Pulmonary Atresia With Intact Ventricular Septum

Kelli GriffithsUndergraduate StudentUniversity of Florida

Redmond P. Burke MDChief, Division of Cardiovascular SurgeryThe Congenital Heart InstituteMiami Childrens Hospital 2014

Danny Monroe, RNDanielle Madril, MDRichard Lagueruela, MD

FRANCISCO ALONSO, RNFAChristopher Tirotta MD

Pulmonary Atresia with Intact Ventricular Septum: PA/IVSIncidenceRepresents 1-1.5% of all congenital heart diseasePA/IVSis a uniformly fatal form of structural cardiac disease. Outcomes of surgical interventions are improving with a five-year survival rate of approximately 80 percent

5MorphologyMarked morphologic heterogeneity is the hallmark of this lesionHeterogeneity plus rarity produces uncertainty in therapeutic planningPulmonary ValvePlate-like valveLong segment stenosisPatent Foramen OvaleTrue ASD in 20%5-10% restrictiveRight AtriumDilated and proportional to the amount of TR

MorphologyTricuspid valveUsually smaller than normal but ranges from Ebsteins anomaly (5-10%) with enlarged or dilated annulus to extremely stenotic valveMedian Z values around -2Correlates with size of RVVarying types of dysplasia are seen

MorphologyRight VentricleHypertrophied with reduced cavity in 90% and of these, 60% are severeCan be Unipartite: inlet part onlyBipartite: inlet and outletTripartite: inlet, outlet, trabecularFibrosis can be present and sometimes severe in both ventricles

Morphology Coronary arteriesFistulae between RV sinusoids and coronary arteries are seen in 8-55% of small hypertensive RVsCorrelates with TV diameter, RV size and degree of TRMore often LAD and LCA than RCA20% have RV dependent coronary circulationRVDCCMorphologySinusoidsCommunications between coronary artery system and the RV through endothelial lined blind channelsPath of flow is from the RV through the intramyocardial sinusoids to the coronary arteries to coronary veins through the coronary sinus to the RA and back to the RVCoronary flow can be easily compromised if RV systolic pressure is reduced by any means

In 2009, we reviewed our Midterm results for Pa/IVS at MCHRetrospective study from 1996 to 200724 PatientsMean age at first intervention 4.5 daysMean follow-up of 6.05 years (range 1.9 to 12.7 years)

Ann Thorac Surg. 2009 Apr;87(4):1227-33.Midterm results for collaborative treatment of pulmonary atresia with intact ventricular septum.Hannan RL1,Zabinsky JA,Stanfill RM,Ventura RA,Rossi AF,Nykanen DG,Zahn EM,Burke RP.

PA/IVS treatment plans are based on Echo and Catheterization, followed by presentation at team conferenceHistorically, a wide range of surgical options have been used for this heterogeneous group of patients.We have adopted a combined surgical and catheter-based approachInitial treatment is dictated by the size and morphologic development of the RV, the RVOT, the TV and by the presence or absence of coronary abnormalities.

Tricuspid valve Z-score is a Critical Determinant

Coronary artery anatomy is another critical determinant

63% of our patients had severe TV or RV hypoplasia or RVDCC, and were placed in the Single Ventricle PathwayStrategy to reduce cumulative traumaWe try to avoid bypass and ischemic arrestComplete palliation in cath labBalloon Atrial septostomy and Ductal stentChallenges:Incomplete septostomyTortuous ductHemodynamic instability with wire across TVArrhythmia and TR

Balloon atrial septostomy in cath lab and Central shunt or RMBTS off pumpChallenges:Inadequate septostomyShunt thrombosis/bleeding/hemodynamic instability from clamps on PAPulmonary artery ductal stenosisTime from cath Intervention to surgery

Incomplete Septostomy

Floppy edge of septum primum

Post Surgical Septectomy

TORTUOUS DUCT

Tortuous DuctCentral Shunt Proximal anastomosis

Thrombosed central shunt

Tenacious thrombus resists thrombectomy and requires a new shunt

Innovations to reduce surgical traumaBiological glues for shunt suture lines.Veno-venous bypass support circuitsRotational thromboelastometry (ROTEM) analysis of postoperative coagulopathyEcho guided surgical septostomy with endoscopic instruments passed through the atrial wall in beating heart, on or off bypass, in cath lab or in ORAttempted twice, but difficult to achieve good septostomy with current instrumentation

37% of our patients with PA/ivs were placed in the 2 ventricle pathwayMild or moderate RV hypoplasiaPlate-like pulmonary atresiaNo RVDCC

We attempt to use the Least Traumatic Approach for each patientTranscatheter RF perforation of the valve plate and ballooon valvuloplasty with surgical standbyHybrid approach via subxyphoid incisionTransannular right ventricular outflow tract patch, with or without shunt

Hybrid ventricular decompression in pulmonary atresia with intact septum Burke RP, Hannan RL, Zabinsky JA, Tirotta CF, Zahn EM. Ann Thorac Surg. 2009 Aug;88(2):688-9.

Single Ventricle pathShunt alone and single ventricle repair expectedCath in 3-6 mos. with plan of separating circulationsGlenn done at 3-12 mos. Warm and beating heartBetween 1-4 years convert to a modification of the FontanTotal cavopulmonary reconstruction if RV dep. coronary circulationFontan is done with heart warm and beating

Two or 1.5 Ventricle Path If RVOT Procedure alone or with shuntCath and echo in 3-6 mos to evaluate growth of RV and TVIn cath lab, attempt temporary closure of shunt and ASD and if adequate response, proceed to permanent closureIf no growth, switch to single ventricle pathwayIf borderline, repeat cath and echo in 1 yearMiami Childrens ExperiencePulmonary Valve PerforationAnd Valvuloplasty (10)BTS or Central Shunt (7)BDCPA (3)Alive (3)Alive (3)Alive (4)2V PathwayGroup A (37%)BTS or Central Shunt (13)BDCPA (9)Death (3)Fontan (1)Alive (2)Fontan (7)Alive (7)Cath Septostomy (4)Death (1)Fontan (2)Alive (2)Alive (1)BDCPA (1)Cath Septostomy (1)BTS (4)BDCPA (3)1V PathwayGroup B (63%)Alive (1)There was no crossover between the Two ventricle repair group, and the Single ventricle group.Survival

PrognosisMCH91.7% survival at 6 years (22/24)CHSS81% survival at 1 month64% survival at 4 yearsMayo Clinic Adult Congenital Heart ClinicTwenty adult patients with PA/IVS (1998 to 2009)Median age at death was 32 years (30-37 years)Seven patients underwent the Fontan operation, eight patients had a biventricular repair, and five patients remained with palliative shuntsAll patients required re-interventions in adulthood. Tricuspid valve (TV) (n=5), pulmonary valve (PV)/conduit (n=6), and mitral valve (n=2) replacements were the most frequent Atrial arrhythmias were present in 80% of the total cohortFuture DevelopmentsFetal echo and intervention

ConclusionsAn individualized, collaborative approach to PA/IVS can produce good results.If the right ventricle can be safely decompressed and looks usable, the need for a shunt after valvuloplasty does not preclude a two or 1.5 ventricle repair.Anatomy mandating a neonatal shunt has substantial early mortality.Hybrid procedures may help reduce the cumulative trauma of care for this complex lesion.Thank You