Importance of surgical expertise in septal myectomy for obstructive hypertrophic cardiomyopathy.

OBJECTIVE: In 2011, a multidisciplinary hypertrophic cardiomyopathy (HCM) program with a dedicated myectomy surgeon was implemented at our institution. We hypothesized that a dedicated approach allows better identification and management of mitral regurgitation (MR) during septal myectomy (SM) for obstructive HCM with significant mitral regurgitation. METHODS: Between 2006 and 2018, 181 patients had SM at our institution. This study consists of 53 patients with preoperative moderate or greater MR associated with systolic anterior motion who underwent isolated SM with or without mitral intervention. Patients were divided into those who underwent SM by a dedicated myectomy surgeon (group D, n = 31) or by a non-dedicated surgeon (group ND, n = 22). Primary outcome of interest was rate of mitral valve replacement (MVR) at SM. Secondary outcomes include in-hospital mortality, need for permanent pacemaker, mitral valve reoperation, and residual MR and left ventricular outflow tract gradient on postoperative echocardiography. RESULTS: 12 patients (55%) had a concomitant MVR during septal myectomy in group ND compared to 2 patients (6%) in group D (p < 0.01). Among patients who did not undergo MVR, patients in group D less commonly had residual MR than patients in ND after SM (p < 0.01). Group D had 100% survival with NYHA class I in 94% patients at follow-up visit (p = 0.01). Reoperation for MVR was required in four patients in group ND vs. none in group D (p < 0.01). CONCLUSIONS: A dedicated surgeon is able to spare the mitral valve in patients undergoing SM. This study emphasizes the importance of surgical expertise in this cohort.

Heart failure in adult congenital heart disease: tetralogy of Fallot.

The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication. Its occurrence is strongly associated with adverse outcome. Unfortunately, conventional concepts of heart failure may not be directly applicable in this patient group. This article presents a review of the current knowledge on HF in rTOF patients, including incidence and prevalence, the most common mechanisms of heart failure, i.e., valvular pathologies, shunt lesions, left atrial hypertension, primary left heart and right heart failure, arrhythmias, and coronary artery disease. In addition, we will review information regarding extracardiac complications, risk factors for the development of heart failure, clinical impact and prognosis, and assessment possibilities, particularly of the right ventricle, as well as management strategies. We explore potential future concepts that may stimulate further research into this field.

Virtual septal myectomy for preoperative planning in hypertrophic cardiomyopathy.

OBJECTIVE: Although septal myectomy (SM) is the preferred treatment for medication-refractory obstructive hypertrophic cardiomyopathy, the procedure remains subjective. We have developed a virtual myectomy (VM) technique using 3-dimensional reconstruction of gated cardiac computed tomography (CT) to assist intraoperative objective assessment of the adequacy of the resection. METHODS: We retrospectively reviewed patients 15 patients who underwent a SM guided by preoperative VM at our program between March 2016 and July 2017. Gated cardiac CT was performed to allow delineation of the left ventricular (LV) myocardium at end-diastole to replicate the cardioplegic myocardial arrest (90%-95% RR interval). SM was performed to attain resection volume predicted by VM. Retrospective, blinded VM also was performed with fixed parameters to determine relationship between ideal (VM1) and conservative (VM2) VM and actual resection. RESULTS: Mean patient age was 52.1 ± 10.6 years, 27% were male, and 80% had New York Heart Association class 3 or 4. Preoperative mean peak LV outflow tract gradient was 79 mm Hg (range 47-82). In-hospital mortality was 0%. Mean postoperative LV outflow tract gradient was 13 mm Hg (11-19). Gated cardiac CT was performed with mean phase 94% (86%-98%). Mean total LV myocardial volume was 226 cm3 (146-365) and volume of the asymmetric portion of the LV was 19 cm3 (5.2-48.8). Actual surgical resection volume was 6.2 ± 1.7 cm3. Retrospective VM1 and VM2 performed postoperatively blinded to surgical results were 12.8 cm3 (4.8-29.23) and 6.7 cm3 (3.5-13.2), showing a modest correlation (R1 = 0.44, R2 = 0.56) with actual myectomy. CONCLUSIONS: Three-dimensional CT and VM can be a viable addition to preoperative assessment of patients with obstructive hypertrophic cardiomyopathy.

Multi-Modality Imaging in the Evaluation and Treatment of Tricuspid Regurgitation.

PURPOSE OF REVIEW: The goal of this review is to cover the epidemiology of tricuspid regurgitation (TR), anatomy of the tricuspid valve (TV), and the mechanisms and modern treatment of TR. The focus will be on the role of echocardiography, cardiac CT, and MRI to determine the mechanism, severity, and management strategies of TR. RECENT FINDINGS: The evaluation and management of TR is a rapidly growing field with significant advances in both imaging and interventions. Important advances have been made to understand TV anatomy and physiology in 3D echo, CT, and MRI. Additional understanding of the abnormal outcomes in both primary TR and secondary TR have been appreciated. Multiple transcatheter devices have reached the stage of early trials in high surgical risk cohorts with favorable initial findings. TR is a significant cardiovascular problem and vastly undertreated in the present era. There has been tremendous growth in knowledge of mechanisms of TR, its prognostic implications, timing of intervention, and development of novel treatment strategies. Multimodality imaging plays a key role in evaluation and treatment of this condition.

Role of computed tomography angiography for HeartMate II left ventricular assist device thrombosis.

INTRODUCTION: Device thrombosis is one of the most devastating complications of HeartMate II left ventricular assist devices. The purpose of this study is to assess the anatomical properties that the inflow and outflow computed tomography angiography provides in assessing for left ventricular assist device thrombosis, as well as their impact on clinical management and postoperative outcomes. METHODS: Between April 2010 and December 2016, 22 patients who received a HeartMate II left ventricular assist device implantation were readmitted for suspected device thrombosis and underwent a computed tomography angiography for workup. Left ventricular assist device-associated anatomy was assessed, including outflow abnormality on computed tomography angiography (contrast filling defect), inflow abnormalities on computed tomography angiography (space at inflow, M-I angle), and inflow abnormalities on chest X-ray (inflow angulation, pump pocket depth). RESULTS: Computed tomography angiography revealed an outflow filling defect in three patients (14%) resulting in change in surgical approach from subcostal pump exchange to resternotomy pump and outflow graft exchange. Inflow graft malpositioning was identified in four patients, with the inflow abutting the left ventricular wall and obstructing the cannula opening. On computed tomography angiography assessment, mean space at inflow was 5.3 ± 1.6 mL and M-I angle was 35.6° ± 6.6°. Chest X-ray evaluation revealed mean inflow angle and pump pocket depth of 75.7° ± 13.4° and 110.2 ± 26.6 mm, respectively. CONCLUSION: Computed tomography angiography provides a noninvasive assessment of the outflow graft and inflow cannulas in left ventricular assist device patients. Findings on computed tomography angiography reveal possible mechanical etiologies of thrombosis and may be useful for determining the surgical management of device thrombosis patients.

Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis.

BACKGROUND: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. METHODS: Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively. The study protocol IRB-AAAO1805 was approved. RESULTS: Forty-seven patients were identified, including 20 infants with significant associated congenital heart defects (42.6%, including 7 with single ventricle physiology), and 10 infants (21.3%) and 16 noninfants (34.0%) with isolated scimitar syndrome. Median follow-up was 3.55 years. Noninfants exhibited lower incidences of right pulmonary artery hypoplasia (p < 0.001), aortopulmonary collaterals (p = 0.004), and scimitar vein obstruction at the caval confluence (p = 0.032). Eighteen patients (38.3%) underwent surgical repair for scimitar syndrome. Overall mortality after baffle repair or scimitar vein reimplantation was 37.5% (3 of 8) for infants and 0% (0 of 6) for noninfants (p = 0.209). Overall mortality for medically managed infants was 46.7% (7 of 15) compared with 0% (0 of 8) for noninfants (p = 0.052). Multivariable analyses identified infantile onset as an independent risk factor for stenosis or obstruction after repair (hazard ratio 9.34, p = 0.048), and single ventricle physiology as an independent risk factor for mortality among unrepaired patients (hazard ratio 29.8, p = 0.004). CONCLUSIONS: The severity of scimitar syndrome depends on presenting age and associated congenital heart disease. Nonsurgical and surgical outcomes are suboptimal for infantile disease, which is a risk factor for stenosis after repair. Single ventricle physiology is associated with poor prognosis.

Postoperative tricuspid regurgitation after adult congenital heart surgery is associated with adverse clinical outcomes.

OBJECTIVE: Many patients with adult congenital heart disease will require cardiac surgery during their lifetime, and some will have concomitant tricuspid regurgitation. However, the optimal management of significant tricuspid regurgitation at the time of cardiac surgery remains unclear. We assessed the determinants of adverse outcomes in patients with adult congenital heart disease and moderate or greater tricuspid regurgitation undergoing cardiac surgery for non-tricuspid regurgitation-related indications. METHODS: All adult patients with congenital heart disease and greater than moderate tricuspid regurgitation who underwent cardiac surgery for non-tricuspid regurgitation-related indications were included in a retrospective study at the Schneeweiss Adult Congenital Heart Center. Cohorts were defined by the type of tricuspid valve intervention at the time of surgery. The primary end point of interest was a composite of death, heart transplantation, and reoperation on the tricuspid valve. RESULTS: A total of 107 patients met inclusion criteria, and 17 patients (17%) reached the primary end point. A total of 68 patients (64%) underwent tricuspid valve repair, 8 patients (7%) underwent tricuspid valve replacement, and 31 patients (29%) did not have a tricuspid valve intervention. By multivariate analysis, moderate or greater postoperative tricuspid regurgitation was associated with a hazard ratio of 6.12 (1.84-20.3) for the primary end point (P = .003). In addition, failure to perform a tricuspid valve intervention at the time of surgery was associated with an odds ratio of 4.17 (1.26-14.3) for moderate or greater postoperative tricuspid regurgitation (P = .02). CONCLUSIONS: Moderate or greater postoperative tricuspid regurgitation was associated with an increased risk of death, transplant, or reoperation in adult patients with congenital heart disease undergoing cardiac surgery for non-tricuspid regurgitation-related indications. Concomitant tricuspid valve intervention at the time of cardiac surgery should be considered in patients with adult congenital heart disease with moderate or greater preoperative tricuspid regurgitation.

Adult congenital heart disease and pregnancy.

Adults with congenital heart disease now form the largest group of women with cardiac disease becoming pregnant in the developed world. This is both a mark of impressive steps forward in the management of congenital heart disease and also a challenge to the medical community to develop systems of care that will best serve these women and their babies. Each woman with congenital heart disease presents a unique pattern of challenges for the cardiologist, obstetrician, and anesthesiologist, and their care should be tailored to deal with their individual circumstances. As this population of patients continues to grow, we must continue to learn and improve our diagnostic tools and management strategies to refine their care. This review intends to focus on reviewing the outcomes in this set of patients and also an approach to the assessment and the management of these patients, primarily for an audience of obstetricians, pediatricians, and anesthesiologists.