Long-Term Follow-Up in Adult Survivors of Pulmonary Atresia With Intact Ventricular Septum

Bui, Christine; Lam, Wilson; Franklin, Wayne; Ermis, Peter

doi:10.1016/s0735-1097(17)33956-6

Cited by 2 publications

(2 citation statements)

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“…Although the survival rates of patients with PA-IVS have substantially increased with the majority of deaths occurring within the first postoperative year, and 15-year survival rates of all subtypes of PA-IVS patients being reported to be anywhere from 58% to 87%, 4,40 many patients living into adulthood may require re-intervention or succumb to late-onset arrhythmias. [61][62][63] Conclusion PA-IVS clearly comprises a heterogeneous group of patients, which requires accurate morphological and functional assessment to appropriately prognosticate and dichotomize into a biventricular repair versus univentricular palliative strategy. The preoperative management requires a thorough understanding of single ventricle physiology, including the necessity for unrestrictive atrial communication and a patent arterial duct.…”

Section: Long-term Follow-upmentioning

confidence: 99%

Section: Long-term Follow-upmentioning

confidence: 99%

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Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Chikkabyrappa

Loomba

Tretter

2018

Semin Cardiothorac Vasc Anesth

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Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle-dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management.

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Section: Long-term Follow-upmentioning

confidence: 99%