ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common genetic or developmental factors, or some combination requires further investigation.

BackgroundExisting data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D‐transposition of the great arteries (D‐TGA) are heterogeneous and limited by statistical power.Methods and ResultsWe conducted a systematic review and meta‐analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow‐up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter‐defibrillator therapy in this population. Average survival dropped to 65% at 40 years after atrial switch repair, with sudden cardiac death accounting for 45% of all reported deaths. Mortality was significantly lower in cohorts that were more recent and operated on younger patients. Patient‐level risk factors for late mortality were history of supraventricular tachycardia (odds ratio [OR] 3.8, 95% CI 1.4–10.7), Mustard procedure compared with Senning (OR 2.9, 95% CI 1.9–4.5) and complex D‐TGA compared with simple D‐TGA (OR 4.4, 95% CI 2.2–8.8). Significant risk factors for sudden cardiac death were history of supraventricular tachycardia (OR 4.7, 95% CI 2.2–9.8), Mustard procedure (OR 2.2, 95% CI 1.1–4.1), and complex D‐TGA (OR 5.7, 95% CI 1.8–18.0). Out of a total 124 implantable cardioverter‐defibrillator discharges over 330 patient‐years in patients with implantable cardioverter‐defibrillators for primary prevention, only 8% were appropriate.ConclusionsPatient‐level risk of both mortality and sudden cardiac death after atrial switch repair are significantly increased by history of supraventricular tachycardia, Mustard procedure, and complex D‐TGA. This knowledge may help refine current selection practices for primary prevention implantable cardioverter‐defibrillator implantation, given disproportionately high rates of inappropriate discharges.

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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 AE 10.6 years, 27% were male, and 80% had New York Heart Association class 3 or 4. Preoperative mean peak LVoutflow tract gradient was 79 mm Hg (range 47-82). In-hospital mortality was 0%. Mean postoperative LVoutflow tract gradient was 13 mm Hg (11)(12)(13)(14)(15)(16)(17)(18)(19). Gated cardiac CT was performed with mean phase 94% (86%-98%). Mean total LV myocardial volume was 226 cm 3 (146-365) and volume of the asymmetric portion of the LV was 19 cm 3 (5.2-48.8). Actual surgical resection volume was 6.2 AE 1.7 cm 3 . Retrospective VM1 and VM2 performed postoperatively blinded to surgical results were 12.8 cm 3 (4.8-29.23) and 6.7 cm 3 (3.5-13.2), showing a modest correlation (R 1 ¼ 0.44, R 2 ¼ 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.

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D-TGA is a form of cyanotic congenital heart disease (CHD) representing ~ 3% of all CHD and almost 20% of all cyanotic CHD. Since the late 1980s, standard of care is to repair these patients with an arterial switch operation (ASO) as opposed to a Mustard/Senning operation. The long-term survival and complication rates are superior in the ASO. Long-term follow-up is recommended for all D-TGA patients and includes management with adult congenital heart disease specialists and the use of echocardiography and advanced imaging with CT or MRI. The most common complications seen are pulmonary stenosis, coronary artery stenosis, and neo-aortic regurgitation. Careful evaluation of new symptoms or declining function is essential in preventing and treating these long-term sequelae.