Left ventricular inflow obstruction associated with persistent left superior vena cava and dilated coronary sinus

AbstractBackgroundIt has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition.MethodsSince 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial “a” waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients.ResultsThere were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient.ConclusionsWe conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique

Pediatric ventricular assist device use as a bridge to transplantation does not affect long-term quality of life

ObjectiveThe present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL.MethodsThe present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated.ResultsAt follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the survey's Core Module, nor were any differences found in the outcomes assessed using the survey's Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores.ConclusionsOver the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation

Cor Triatriatum Sinister With an Intact Interatrial Septum and a Decompressing Vein in a Toddler

<p><strong>Objectives</strong></p><p>Cor triatriatum sinister is a very rare cardiac anomaly that may lead to pulmonary hypertension, right ventricular dilation, and eventually right heart failure.</p><div><p><strong>Methods</strong></p><p>The authors report a procedure performed for a toddler who was misdiagnosed with asthma as an infant. She had progressive respiratory symptoms and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, severe tricuspid regurgitation, and severe right ventricular dysfunction.</p><p><strong>Results</strong></p><p>The surgical repair, shown in the video, was uneventful and the right ventricular function was normalized on follow-up echocardiograms.</p><p><strong>Conclusion</strong></p><p>To the authors' knowledge, this is the first report of cor triatriatum sinister with a restrictive fenestration, a decompressing vertical vein, an intact interatrial septum, and severe tricuspid regurgitation in a toddler. The treatment was successful as demonstrated by the follow-up echocardiograms, which show normalization of the ventricular function and pulmonary artery pressure.</p><p>This educational content was originally presented during the STSA 64th Annual Meeting. This content is published with the permission of the <a href="https://stsa.org/">STSA</a>. For more information on the STSA and its next Annual Meeting, please click <a href="http://stsa.org/annualmeeting/">here</a>.</p></div