Improved results with selective management in pulmonary atresia with intact ventricular septum

Jahangiri, Marjan; Zurakowski, David; Bichell, David P.; Mayer, John E.; Nido, Pedro J. del; Jonas, Richard A.

doi:10.1016/s0022-5223(99)70100-5

Cited by 107 publications

(101 citation statements)

References 17 publications

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“…10 In a highly selective patient population, 98% survival can be achieved. 11 Final-stage surgery for these patients incorporates all potential circulations, including a biventricular or 1 1 ⁄2-ventricle repair 2 or univentricular palliation. 12 The choice of treatment algorithm depends on the estimated adequacy of the RV to cope with systemic venous return, the size of tricuspid valve, and the status of the coronary arteries.…”

Section: Discussionmentioning

confidence: 99%

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

et al. 2003

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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 ½-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.

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Section: Discussionmentioning

confidence: 99%

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

et al. 2003

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“…53,54 Lower mortality in the current era may reflect (1) better selection of patients to decompress and attempt a biventricular repair, (2) the recognition of overcirculation and coronary steal in the initial palliation, and (3) the incorporation of transplantation as one of the therapeutic options. [55][56][57][58] With the overall survival having improved, the most practical approach may be to study the coronary circulation on diagnosis of those with a small tricuspid valve annulus, because it is now apparent that an RV-dependent coronary circulation typically exists in the hypoplastic RV unsuitable for biventricular repair. If an RV-dependent coronary circulation exists in an asymptomatic patient with normal left ventricular function and wall motion, then a reasonable option is to proceed with palliative surgery with a systemic-to-PA shunt with postoperative management that minimizes overcirculation, followed by early placement of a bidirectional cavopulmonary shunt and a Fontan procedure.…”

Section: Other High-risk Chd Lesionsmentioning

confidence: 99%

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Ross¹,

Law²,

Book³

et al. 2016

Circulation

118

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“…293 Patients with pulmonary atresia and intact ventricular septum with RV-dependent coronary circulation may have a higher risk of death resulting from palliation, especially after an aortic-to-pulmonary shunt. [294][295][296][297][298][299] Transplantation can be considered in these patients. Patients with SV and heterotaxy syndrome do not seem to have a longterm outcome that is as good as for other SV patients.…”

Section: Pediatric-specific Issuesmentioning

confidence: 99%

Chronic Heart Failure in Congenital Heart Disease

Stout¹,

Broberg²,

Book³

et al. 2016

Circulation

276

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Improved results with selective management in pulmonary atresia with intact ventricular septum

Abstract: 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.

Cited by 107 publications

References 17 publications

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Chronic Heart Failure in Congenital Heart Disease

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