Hybrid Approach for Pulmonary Atresia with Intact Ventricular Septum: Early Single Center Results and Comparison to the Standard Surgical Approach

Catheterization and Cardiovascular Interventions 83:753–761 (2014) PEDIATRIC AND CONGENITAL HEART DISEASE Original Studies Hybrid Approach for Pulmonary Atresia With Intact Ventricular Septum: Early Single Center Results and Comparison to the Standard Surgical Approach 1 2 3 Jeffrey D. Zampi, * MD, Jennifer C. Hirsch-Romano, MD, MS, Bryan H. Goldstein, MD, 4 1 Justin A. Shaya, and Aimee K. Armstrong, MD Objectives: To examine acute and mid-term patient outcomes following the hybrid approach to pulmonary atresia with intact ventricular septum (PA-IVS) compared with the standard surgical approach. Background: A subset of PA-IVS patients with the prospect of biventricular circulation typically undergo surgical or transcatheter right ventricular (RV) outflow tract opening. A recently described hybrid procedure, involving perventricular pulmonary valve perforation, was shown to be safe and effective in single-center series. Methods: A single-center retrospective review of all patient with PA-IVS who underwent either surgical or hybrid RV decompression between January 2002 and De- cember 2011 was completed and acute and mid-term patient outcomes were compared between the surgical and hybrid cohorts. Additionally, a systematic literature review was completed to compare a transcatheter cohort to the hybrid cohort. Results: Seven patients with PA-IVS underwent a hybrid procedure; the procedure was technically suc- cessful in all attempts, and none required CPB. No patients required surgical re- intervention prior to hospital discharge, and none died during the study period. Surgical RV decompression was performed in 17 patients with a median CPB time of 80 min. Patient outcomes were nearly identical between cohorts. By systematic review, the transcatheter approach has a procedural success of 75–95% but up to 75% of patients require operation in the neonatal period. Conclusions: The hybrid approach is a safe and feasible alternative to the standard surgical and transcatheter approaches to PA-IVS. Acute and mid-term patient outcomes are comparable with those treated with a standard surgical approach and neonatal CPB is completely avoided. VC 2013 Wiley Periodicals, Inc. Key words: congenital heart defect; patient outcome assessment; pediatric interventions; perventricular procedure; hybrid cardiac surgery INTRODUCTION disease with significant morphologic heterogeneity. Pulmonary atresia with intact ventricular septum Because of this heterogeneity, repair or palliation strat- (PA-IVS) is a rare form of complex congenital heart egies vary widely, ranging from single ventricle 1Division of Pediatric Cardiology, Department of Pediatrics, *Correspondence to: Jeffrey D. Zampi, University of Michigan University of Michigan, Ann Arbor, Michigan Congenital Heart Center, C.S. Mott Children’s Hospital, Floor 11, 2Division of Pediatric Cardiac Surgery, Department of Surgery, Cardiology, 1540 E. Hospital Drive, Ann Arbor, MI 48109-4204. University of Michigan, Ann Arbor, Michigan E-mail: [email protected] 3The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio Received 24 June 2013; Revision accepted 25 August 2013 4University of Michigan Medical School, Ann Arbor, Michigan DOI: 10.1002/ccd.25181 Conflict of interest: Nothing to report. Published online 31 August 2013 in Wiley Online Library (wileyonlinelibrary.com) VC 2013 Wiley Periodicals, Inc. 754 Zampi et al. palliation to complete biventricular repair. Overall, with PA-IVS who underwent an RV decompression regardless of anatomical subtype, survival is poor, procedure between January 2002 and December 2011. reported to be as low as 50% at 5 years [1–5]. For Patients were identified using a combination of our in- patients with anatomy favorable for a biventricular ternal surgical database (LUMEDX Corporation, Oak- repair (i.e., adequate tricuspid valve, adequate right land, CA) and by performing a keyword search in our ventricle, and no evidence of right ventricular depend- electronic medical record system (CareWeb, non- ent coronary circulation), the initial procedural focus is commercial, live since 1998) for the terms “pulmonary on creation of a patent right ventricular outflow tract atresia” and “intact ventricular septum.” Medical re- (RVOT) to decompress the right ventricle and to allow cord review was completed to ensure only patients for anterograde pulmonary blood flow. This procedure who underwent RV decompression as the initial surgi- may be performed surgically or percutaneously and, in cal repair were included in the analysis. At our institu- select patients, might result in growth of the right ven- tion, the hybrid approach to PA-IVS was instituted in tricle [6,7] with the ability to proceed to a biventricular October 2010; previously, the surgical approach was circulation [1,2]. Both techniques, however, have the treatment of choice and subsequently, only 2 limitations and risks. The surgical approach requires a patients were managed with the surgical approach. sternotomy and cardiopulmonary bypass (CPB) and has Patients who underwent a hybrid procedure for PA- 1- and 5-year survival rates of 70–75% and 60–67%, IVS were identified by search of the institutional respectively [1–3]. With risk-stratification based on hybrid procedure database. Data were collected on all several morphologic criteria, however, the mid-term patients to assess baseline demographic information, survival rate was as high as 98% in one series [5]. The pre-operative procedural risk factors and echocardio- percutaneous transcatheter approach, which involves graphic assessment of cardiac structure and function, pulmonary valve perforation (via directed stiff guide- as well as post-procedural patient outcomes during the wire, laser-assisted, or radiofrequency-assisted perfora- initial hospitalization and for 2 years following the pro- tion) and balloon valvuloplasty, avoids sternotomy and cedure. Whenever possible, all echocardiographic CPB but has a variable rate of procedural failure (5– assessments, measurements and grading were per- 25%), a significant risk of cardiac perforation (5–50%), formed by the core Pediatric Cardiology Echocardiog- a procedural mortality of up to 17%, and hospital mor- raphy Laboratory at our institution. tality of up to 50% [6–14]. In addition, patients treated Normally distributed variables were compared using with the percutaneous approach have a significant risk Students t-test. Chi square was used to compare cate- (up to 76%) of requiring surgical intervention in the gorical variables, and nonparametric, ordinal variables neonatal period either to augment pulmonary blood were compared using Wilcoxon Rank Sum. A P value flow or to repair a complication associated with the of < 0.05 was considered statistically significant. initial transcatheter procedure [4,6,7,9–13]. A systematic review of the literature was performed Recently, a hybrid approach combining surgical to examine patient outcomes after the percutaneous access with a transcatheter intervention to open the transcatheter treatment of PA-IVS. Using key word RVOT was described [15]. Potential advantages of the searches for the following terms and combination of hybrid approach include avoidance of neonatal CPB, terms: “pulmonary atresia,” “intact ventricular septum,” mitigation of the technical failure rate of the percutane- “perforation,” “transcatheter,” and “percutaneous,” we ous approach, due to direct access to the RVOT, and a searched the English language literature with PubMed lower complication rate (i.e., no risk of RVOT perfora- and OVID databases for all studies published between tion and associated sequelae) [16–19]. While the 1997 and 2012. Inclusion criteria for the literature hybrid balloon pulmonary valvuloplasty procedure has search were single center studies, multi-center studies, been detailed in limited case reports and a single- and published systematic reviews or meta-analyses center series, it has yet to be compared rigorously with which focused on patient outcomes. Exclusion criteria a contemporaneous cohort treated with the standard were case reports or duplications of previously pub- surgical approach. We sought to compare acute and lished data. All references were evaluated from the mid-term outcomes following the hybrid approach to manuscripts to confirm inclusion of all pertinent stud- the standard surgical approach in initial treatment of ies. Specific data collected during the systematic pulmonary atresia with intact ventricular septum. review included rates of procedural success/failure, procedural complications including RVOT perforations and mortality, need for subsequent cardiac operation(s), METHODS and rate of achieving an eventual biventricular circula- After obtaining institutional review board approval, tion. Descriptive comparison between the percutaneous we performed a single-center review of all patients transcatheter and hybrid cohorts was performed. Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI). Hybrid Approach to PA-IVS 755 Hybrid Procedural Description Hospital Outcomes A midline sternotomy is performed and the pericar- There were no significant differences between dium suspended. Circumferential control of the ductus cohorts in the maximum vasoactive-inotropic score arteriosus is obtained. Using transesophageal echocardio- (VIS), duration of mechanical ventilation, length of in- graphic (TEE) guidance exclusively, a

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