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Roosevelt Bryant, III
Edward R. Nowicki
Brian W. Duncan
Muhammad Mumtaz
Eugene H. Blackstone
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Right arrow Congenital - cyanotic

J Thorac Cardiovasc Surg 2008;136:735-742
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum

Roosevelt Bryant, III, MDa, Edward R. Nowicki, MDa, Roger B.B. Mee, MB, ChB, FRACSb, Jeevanantham Rajeswaran, MScc, Brian W. Duncan, MDd, Geoffrey L. Rosenthal, MD, PhDb, Uthara Mohan, MDd, Muhammad Mumtaz, MDb, Eugene H. Blackstone, MDa,c,*

a Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio
b Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, Ohio
c Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, Ohio
d Department of Pediatric Cardiology, Cleveland Clinic, Cleveland, Ohio

Received for publication July 3, 2007; revisions received February 27, 2008; accepted for publication March 30, 2008.

* Address for reprints: Eugene H. Blackstone, MD, Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, 9500 Euclid Ave/Mail Stop JJ-40, Cleveland, OH 44195. (Email: blackse{at}ccf.org).

Objectives: Right ventricular sinus myectomy has been proposed for pulmonary atresia with intact ventricular septum for morphology falling within the uncertain area for eventual biventricular repair. Our objective was to evaluate right ventricular sinus myectomy by characterizing the morphologic spectrum of these patients, determining whether biventricular repair was achieved, ascertaining growth of right-sided structures, and assessing survival.

Methods: We evaluated medical records, all imaging studies, and follow-up data (complete in all but 1 patient) from 43 patients with pulmonary atresia with intact ventricular septum treated from October 1993 to July 2005, 16 of whom underwent right ventricular sinus myectomy. Serial echocardiographic measurements of right-sided cardiac structures were converted to Z values to estimate their growth relative to somatic growth.

Results: Patients undergoing right ventricular sinus myectomy had mild-to-moderate right ventricular size diminution (grade –1.2 ± 3.2) and a tricuspid valve Z value of –4.9 ± 1.9. Thirteen (87%) of the 16 patients achieved biventricular repair. After right ventricular sinus myectomy, mean right ventricular cavity size grade increased to 1.4 ± 0.66, but the tricuspid valve Z value did not change appreciably over time. Five-year survival after sinus myectomy was 85%; late deaths were in patients with the smallest tricuspid valves at presentation (Z value <–7).

Conclusions: Right ventricular sinus myectomy in the uncertain area for biventricular repair of pulmonary atresia with intact ventricular septum leads to immediate increase in right ventricular volume. It, in combination with establishing right ventricle–pulmonary trunk continuity, allowed early biventricular repair in 87% of patients. However, tricuspid valve growth in relation to somatic growth was minimal. Thus, small tricuspid valve size might limit the long-term success of biventricular repair achieved by means of right ventricular sinus myectomy.

Abbreviations and Acronyms CHSS = Congenital Heart Surgeons' Society; IRB = institutional review board; PAIVS = pulmonary atresia with intact ventricular septum; RV = right ventricular




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