Pulmonary Atresia

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Circulation. 2003 Aug 19;108(7):826-32. Epub 2003 Jul 28.
Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum: impact on patient care.
Humpl T, Soderberg B, McCrindle BW, Nykanen DG, Freedom RM, Williams WG, Benson LN.
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, The University of Toronto School of Medicine, Toronto, ON M5G 1X8, Canada.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve. METHODS AND RESULTS: Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1(1/2)-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time. CONCLUSIONS: Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle-dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.

Journal Thoracic Cardiovascular Surgery 1999;118:1046-1052
IMPROVED RESULTS WITH SELECTIVE MANAGEMENT IN PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM Marjan Jahangiri, FRCS, David Zurakowski, PhD, David Bichell, MD, John E. Mayer, MD, Pedro J. del Nido, MD, Richard A. Jonas, MD From the Department of Cardiac Surgery, Children’s Hospital, Boston, Mass.
Address for reprints: Richard A. Jonas, MD, Department of Cardiac Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115.

Objective: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle–dependent coronary circulation and growth of the right ventricle. Methods: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. Results: Sixteen (34%) patients had a right ventricle–dependent coronary circulation, with a tricuspid valve Z-score of –3.0 ± 0.66 versus –2.0 ± 0.95 (P = .002) for those without a right ventricle–dependent coronary circulation. A systemic–pulmonary artery shunt only was performed in all patients with a right ventricle–dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle–dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle–dependent coronary circulation, 6 patients underwent only a systemic–pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1.5- ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. Conclusions: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.

Journal Thoracic Cardiovascular Surgery 1998;116:924-931
Jack Rychik, MD, Hara Levy, MD, J. William Gaynor, MD, William M. DeCampli, MD, Thomas L. Spray, MD
From the Divisions of Cardiology and Cardiothoracic Surgery, Children's Hospital of Philadelphia, and the Departments of Pediatrics and Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pa.
Read at the Seventy-eighth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 3-6, 1998.
Received for publication May 8, 1998. Revisions requested June 18, 1998.Revisions received Aug 18, 1998. Accepted for publication Aug 18, 1998.
Address for reprints: Jack Rychik, MD, Division of Cardiology, Children's Hospital of Philadelphia, 324 S 34th St, Philadelphia, PA 19104.

Objective:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries. Methods: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular–coronary artery dependency was noted in 8 patients. Results: Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score –2.0 (2.5) vs –2.0 (1.9), P = .83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score –0.53 [1.6], range –3.5 to 1, versus mean Z-score –3.03 [2.7], range –5.5 to 0, P = .002). Conclusions: Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes.

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