Heart Transplantation for Uhl Anomaly in an Adult.

Uhl anomaly is characterized by the morphologic absence of right ventricular myocardium and is an exceedingly rare cause of nonischemic cardiomyopathy. We report the first case of a successful heart transplantation in a 41-year-old patient who presented in cardiogenic shock from Uhl anomaly causing decompensated right ventricular failure.

Long-Term Clinical and Echocardiographic Outcomes Following the Ross Procedure: A Post Hoc Analysis of a Randomized Clinical Trial.

Importance: The Ross procedure as treatment for adults with aortic valve disease (AVD) has been the subject of renewed interest. Objective: To evaluate the long-term clinical and echocardiographic outcomes following the Ross procedure for the treatment of adults with AVD. Design, Setting, and Participants: This post hoc analysis of a randomized clinical trial included adult patients (age <69 years) who underwent a Ross procedure for the treatment of AVD, including those with active endocarditis, rheumatic AVD, decreased ejection fraction, and previous cardiac surgery. The trial, conducted from September 1, 1994, to May 31, 2001, compared homograft root replacement with the Ross procedure at a single center. Data after 2010 were collected retrospectively in November and December 2022. Exposure: Ross procedure. Main Outcomes and Measures: The primary end point was long-term survival among patients who underwent the Ross procedure compared with that in the age-, country of origin- and sex-matched general population. Secondary end points were freedom from any reintervention, autograft reintervention, or homograft reintervention and time-related valve function, autograft diameter, and functional status. Results: This study included 108 adults (92 [85%] male) with a median age of 38 years (range, 19-66 years). Median duration of clinical follow-up was 24.1 years (IQR, 22.6-26.1 years; 2488 patient-years), with 98% follow-up completeness. Of these patients, 9 (8%) had active endocarditis and 45 (42%) underwent reoperations. The main hemodynamic lesion was stenosis in 30 (28%) and regurgitation in 49 (45%). There was 1 perioperative death (0.9%). Twenty-five year survival was 83.0% (95% CI, 75.5%-91.2%), representing a relative survival of 99.1% (95% CI, 91.8%-100%) compared with the general population (83.7%). At 25 years, freedom from any reintervention was 71.1% (95% CI, 61.6%-82.0%); from autograft reintervention, 80.3% (95% CI, 71.9%-89.6%); and from homograft reintervention, 86.3% (95% CI, 79.0%-94.3%). Thirty-day mortality after the first Ross-related reintervention was 0% and after all Ross-related reinterventions was 3.8% (n = 1); 10-year survival after reoperation was 96.2% (95% CI, 89.0%-100%). Conclusions and Relevance: This study found that the Ross procedure provided excellent survival into the third decade postoperatively that was comparable to that in the general population. Long-term freedom from reintervention demonstrated that the Ross procedure may be a durable substitute into late adulthood, showing a delayed but progressive functional decline. Trial Registration: isrctn.org Identifier: ISRCTN03530985.

Cardiac mechanics and reverse remodelling under mechanical support from left ventricular assist devices.

In recent years, development of mechanical circulatory support devices has proved to be a new treatment modality, in addition to standard pharmacological therapy, for patients with heart failure or acutely depressed cardiac function. These include left ventricular assist devices, which mechanically unload the heart when implanted. As a result, they profoundly affect the acute cardiac mechanics, which in turn, carry long-term consequences on myocardial function and structural function. Multiple studies have shown that, when implanted, mechanical circulatory assist devices lead to reverse remodelling, a process whereby the diseased myocardium reverts to a healthier-like state. Here, we start by first providing the reader with an overview of cardiac mechanics and important hemodynamic parameters. We then introduce left ventricular assist devices and describe their mode of operation as well as their impact on the hemodynamics. Changes in cardiac mechanics caused by device implantation are then extrapolated in time, and the long-term consequences on myocardial phenotype, as well as the physiological basis for these, is investigated.

Intracannula Thrombus Formation Associated With Dual Lumen ProtekDuo Cannula in Extracorporeal Membrane Oxygenation (ECMO).

Extracorporeal membrane oxygenation (ECMO) is used in cases of severe respiratory failure refractory to medical management. Use of ECMO is increasing, along with new cannulation strategies including oxygenated right ventricular assist devices (oxy-RVADs). Multiple dual lumen cannulas are now available, which increase the potential for patient mobility and decrease the number of vascular access sites. However, dual lumen, single cannula flow can be limited by adequate inflow, requiring the need for an additional inflow cannula to meet patient demands. This cannula configuration may result in differential flows in the inflow and outflow limbs and altered flow dynamics, increasing the risk of intracannula thrombus. We describe a series of four patients treated with oxy-RVAD for COVID-19-associated respiratory failure complicated by dual lumen ProtekDuo intracannula thrombus.

Robotic and endoscopic mitral valve repair for degenerative disease.

Background: Minimally invasive mitral valve repair has been proven to be a safe alternative to open sternotomy and may be accomplished through classic endoscopic and robotic endoscopic approaches. Outcomes across different minimally invasive techniques have been insufficiently described. We compare early and late clinical outcomes across matched patients undergoing robotic endoscopic and classic endoscopic repair. Methods: From 2011 to 2020, 786 patients underwent minimally invasive mitral surgery, from which we were able to generate 124 matched patients (62 patients in each cohort). Clinical results were then compared between the two matched populations. Survival analysis was used to compare freedom from mortality to 10 years among matched classic endoscopic and robotic endoscopic mitral valve repair cohorts and to calculate freedom from moderate or severe mitral insufficiency at latest follow-up. Histograms of cardiopulmonary bypass (CPB) and aortic cross-clamp times were constructed, and mean bypass and cross-clamp times were compared between classic endoscopic and robotic endoscopic cohorts. Results: There was no difference in early or late mortality at 10 years in either cohort. Freedom from moderate or severe mitral regurgitation or mitral valve replacement at last echocardiogram was 86.4% vs. 73.5% at 10 years, P=0.97. Patients undergoing robotic endoscopic mitral repair had a significantly longer CPB run when compared to the classic endoscopic cohort, with 148 min of CPB in the robotic endoscopic cohort compared to 133 min in the classic endoscopic group, P=0.03. Overall post-operative length of stay was not statistically significant between the robotic endoscopic and classic endoscopic groups, 6.3±0.5 and 6.0±0.3 days, respectively. No patients in either cohort developed renal failure or wound infection. The classic endoscopic group had a slightly higher risk of prolonged ventilation when compared to the robotic endoscopic group, with three classic endoscopic patients remaining intubated >8 hours post-operatively, compared to a single patient in the robotic endoscopic group. There were no unplanned reoperations in either group. Rates of postoperative stroke were comparable between groups (three in the classic endoscopic cohort, and two in the robotic endoscopic cohort). Conclusions: Index mitral valve surgery via a classic endoscopic approach yields similar clinical outcomes when compared to robotic endoscopic surgery. We demonstrate that both classic endoscopic and robotic endoscopic approaches allow repair of degenerative mitral valves with excellent short- and medium-term outcomes in a tertiary referral center.

Optimal Surgical Approach to Left Atrioesophageal Fistula After Catheter Radiofrequency Ablation.

Atrioesophageal fistula formation is a rare but formidable complication after catheter radiofrequency ablation for atrial fibrillation. We present 4 patients who underwent urgent primary intracardiac repair of the left atrium via sternotomy with central cardiopulmonary bypass and early aortic cross-clamp, followed by repair of the esophagus. We believe that this approach represents the safest strategy for addressing this morbid and often fatal complication.

Surgical management of an aneurysmal coronary cameral fistula to the right atrium.

Coronary artery aneurysm and coronary cameral fistula are both often incidentally discovered, and uncommon diagnoses. A coronary artery aneurysm is defined as being 1.5x the size of the adjacent normal coronary artery, while a coronary cameral fistula is a coronary artery that has an abnormal tract directly into a chamber of the heart. In this case report we describe the case of a 72-year-old female who was discovered to have an aneurysmal branch of her right coronary artery fistulized to her right atrium. The fistula had a serpiginous tract and in the midportion of the tract there was a giant aneurysm (3.4 × 3.3 × 3.0 cm) before emptying into the posterior aspect of the right atrium. The aneurysmal aberrant coronary artery was repaired by oversewing the afferent and efferent limbs from the inside. The aneurysm walls were then oversewn. This case demonstrates a unique pathology that was managed with surgical intervention where no standard therapy exists.