Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome
ABSTRACT Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.
This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).
There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.
The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.
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ABSTRACT: Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and diverse form of congenital heart defect. Although most patients with PA/VSD/MAPCAs can wait until they are 3 to 6 months of age to undergo surgical reconstruction, there are three specific criteria that merit an earlier repair. These 3 criteria are (1) unremitting heart failure; (2) a ductus to one lung and MAPCAs to the other; and (3) hemitruncus to one lung and MAPCAs to the other. The purpose of this study was to evaluate our surgical experience with early complete repair of PA/VSD/MAPCAs. This was a retrospective review of patients undergoing complete repair of PA/VSD/MAPCAs within the first 60 days of life. Twenty-seven patients were identified in our database (2002 to 2013) who met these criteria. Fifteen had congestive heart failure, 9 had a ductus plus MAPCAs, and 3 had hemitruncus plus MAPCAs. The median age at surgery was 5 weeks. There was no operative mortality in this cohort of 27 patients. Hemodynamics at the conclusion of the complete repair demonstrated an average right ventricular peak systolic pressure of 32 ± 5 mm Hg and an average right ventricle to aortic pressure ratio of 0.36 ± 0.06. The median length of hospital stay was 26 days. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Eight of the 27 patients have subsequently undergone conduit replacements at our institution. The hemodynamics at the conclusion of the conduit change were statistically unchanged compared with the hemodynamics after complete repair. The data demonstrate that early complete repair of PA/VSD/MAPCAs can be accomplished with low mortality and excellent postoperative hemodynamics. These early hemodynamic results are maintained at medium-term follow-up. We conclude that early complete repair is an appropriate choice for this highly select subgroup of patients.The Annals of thoracic surgery 01/2014; DOI:10.1016/j.athoracsur.2013.10.115 · 3.65 Impact Factor
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ABSTRACT: BACKGROUND: Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA. METHODS: This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation. RESULTS: There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p < 0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p < 0.005). CONCLUSIONS: Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.The Annals of thoracic surgery 02/2013; 95(4). DOI:10.1016/j.athoracsur.2013.01.007 · 3.65 Impact Factor
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ABSTRACT: To determine the effect and safeness of the right ventricle to pulmonary artery connection with occlusion of major aortopulmonary collaterals and pulmonary artery angioplasty to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect beyond the infant period. From December 2009 to August 2012, 37 consecutive patients (mean age 1.9 ± 1.7 years, range 0.6-7.2 years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index 90.9 ± 42.6 mm(2)/m(2); McGoon ratio 1.0 ± 0.2) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, during which most of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was 1.6 ± 0.8 years (range 0.6-3.3 years). Continuous variables are expressed as means ± standard deviation. There were no early deaths, but one patient died of myocarditis 1 year after the rehabilitation. Significant pulmonary artery growth was obtained (Nakata index 215.1 ± 95.1 mm(2)/m(2), P < 0.001; McGoon ratio 1.6 ± 0.5, P < 0.001) in all of the 37 patients, and among them, 17 patients (45.9%) whose pulmonary growth was considered adequate obtained a complete repair without perforation of the ventricular septal defect. The preoperative McGoon ratio might be a good predictor for adequate pulmonary growth. There was one early death after anatomical repair. At the last visit, all survivors who underwent anatomical repair were in New York Heart Association class I-II with satisfactory haemodynamics. Connection of the right ventricle to the pulmonary artery is safe and effective to promote the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, this strategy allows complete repair in the majority of patients beyond infancy.European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 01/2014; DOI:10.1093/ejcts/ezt622 · 2.81 Impact Factor