A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.
OBJECTIVE
To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure.


METHODS
Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed.


RESULTS
Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)].


CONCLUSIONS
Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan.
Yasuhiro
Y
Kotani
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan.
Shingo
S
Kasahara
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan.
Yasuhiro
Y
Fujii
Department of Pediatric Cardiology, Okayama University Hospital, Okayama, Japan.
Takahiro
T
Eitoku
Department of Pediatric Cardiology, Okayama University Hospital, Okayama, Japan.
Kenji
K
Baba
Department of Pediatric Cardiology, Okayama University Hospital, Okayama, Japan.
Shin-Ichi
S
Otsuki
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan.
Yosuke
Y
Kuroko
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan.
Sadahiko
S
Arai
Department of Cardiovascular Surgery, Okayama University Hospital, Okayama, Japan s_sano@cc.okayama-u.ac.jp.
Shunji
S
Sano
D058366:Blalock-Taussig Procedure / Q000379:methods*; D019299:Decompression, Surgical / Q000379:methods*; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome; D054088:Ventricular Septum / Q000000981:diagnostic imaging / Q000601:surgery*
Biventricular repair; Pulmonary atresia and intact ventricular septum; Right ventricle