The Early “Unnatural” History Following Surgical Repair of Ventricular Septal Defects

Introduction Surgical outcomes for simple ventricular septaldefects (VSD) have been excellent in the past three decades. Forthis project, the timing of resolution of left-sided dilation and mitralregurgitation (MR) following VSD repair was assessed. Methods Echocardiographic data surrounding surgery of 42 consecutivechildren who underwent surgical patch repair of a VSD werereviewed. The echocardiograms were reviewed up to a mean of 12months post-operatively (range 9 - 14 months). Quantitative dataindexed to body surface area including left atrial (LA) volume, mitralvalve annulus diameter, and left ventricular end-diastolic dimension(LVEDD) was analyzed. Results The majority of our pre-surgical cohort had only trace(44%) or no MR (31%), with a small proportion having mild (16%)or moderate MR (9%). No patients had moderate or greater MR followingrepair at follow-up. The median mitral valve annular Z-scorewas 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6(range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p< 0.001) at follow-up. LV dilation was present in 81% of pre-operativepatients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9).There was significant improvement in qualitative assessment of LVenlargement (25%) with a median LVEDD Z-score of 0.5 (range:-2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram wasperformed at a mean of 5.7 days (range: 3 - 12 days) following surgery. Conclusions Normalization of LA, mitral valve annulus, and LV sizeoccurred within the first three months in the majority of patients, withsignificant changes occurring within the first post-operative weekfollowing surgical repair for VSD

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

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. </jats:p