NCCM is a heterogeneous condition, and genetic stratification has a role in clinical care. Distinguishing genetic from nongenetic NCCM complements prediction of outcome and may lead to management and follow-up tailored to genetic status.

IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental or familial discovery of IVNC have an encouraging outlook, whereas those who have symptoms of heart failure, a history of sustained ventricular tachycardia or an enlarged left atrium have an unstable course and more severe prognosis.

Background-Left ventricular (LV) noncompaction (LVNC) is a distinct cardiomyopathy featuring a thickened bilayeredLV wall consisting of a thick endocardial layer with prominent intertrabecular recesses with a thin, compact epicardial layer. Similar to hypertrophic and dilated cardiomyopathy, LVNC is genetically heterogeneous and was recently associated with mutations in sarcomere genes. To contribute to the genetic classification for LVNC, a systematic cardiological family study was performed in a cohort of 58 consecutively diagnosed and molecularly screened patients with isolated LVNC (49 adults and 9 children). Methods and Results-Combined molecular testing and cardiological family screening revealed that 67% of LVNC is genetic.Cardiological screening with electrocardiography and echocardiography of 194 relatives from 50 unrelated LVNC probands revealed familial cardiomyopathy in 32 families (64%), including LVNC, hypertrophic cardiomyopathy, and dilated cardiomyopathy. Sixty-three percent of the relatives newly diagnosed with cardiomyopathy were asymptomatic. Of 17 asymptomatic relatives with a mutation, 9 had noncompaction cardiomyopathy. Clinical Perspective on p 239Prevalence of LVNC, estimated from retrospective studies, ranges from 4.5 to 26 per 10 000 adult patients referred for echocardiography. 3-5 LVNC was diagnosed in 3.7% of patients with an LV ejection fraction Յ45%, suggesting that LVNC might not be a rare disorder in adults. 5 In pediatric series, LVNC is the most frequent cardiomyopathy after dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM), comprising Ϸ9% of childhood cardiomyopathies. 6 Clinical features include heart failure, arrhythmias, and thromboembolic events. 3,7 Familial disease was estimated to occur in Ϸ18% to 50% of adults with isolated LVNC, mostly consistent with an autosomal dominant mode of inheritance. 3,8 -13 Intrafamilial phenotypic variability, including LVNC, HCM, and DCM, suggests that these cardio- and rare chromosomal defects and loci. [23][24][25][26][27][28][29][30] The present study investigates the heredity of LVNC, the spectrum of clinical features, and the genetic causes of LVNC by combining systematic cardiological family studies with extensive molecular analysis. Methods Study PopulationThe study comprised 58 unrelated patients with isolated LVNC; 53 were diagnosed consecutively from 2005 to 2008 in the cardiogenetics clinic of the Erasmus MC in Rotterdam and 5 in other tertiary referral centers in The Netherlands. All fulfilled the 4 echocardiographic diagnostic Jenni criteria: (1) excessively thickened LV myocardial wall with a 2-layered structure comprising a compact epicardial layer (C) and a noncompacted endocardial layer (NC) of prominent trabeculations and deep intertrabecular recesses; (2) maximal end-systolic ratio of noncompacted to compacted wall Ͼ2 measured at the parasternal short axis; (3) color Doppler evidence of deep perfused intertrabecular recesses; and (4) absence of coexisting Hoedemaekers et al Family Study of Noncompa...

Although mutations in HCN4 have been previously linked to bradycardia, our study provides the first evidence to our knowledge that mutations in this ion channel gene also may be associated with structural abnormalities of the myocardium.

Summary Solid organ transplant (SOT) recipients may be at risk for severe COVID‐19. Data on the clinical course of COVID‐19 in immunosuppressed patients are limited, and the effective treatment strategy for these patients is unknown. We describe our institutional experience with COVID‐19 in SOT. Demographic, clinical, and treatment data were extracted from the electronic patient files. A total of 23 SOT transplant recipients suffering from COVID‐19 were identified (n = 3 heart; n = 15 kidney; n = 1 kidney‐after‐heart; n = 3 lung, and n = 1 liver transplant recipient). The presenting symptoms were similar to nonimmunocompromised patients. Eighty‐three percent (19/23) of the patients required hospitalization, but only two of these were transferred to the intensive care unit. Five patients died from COVID‐19; all had high Clinical Frailty Scores. In four of these patients, mechanical ventilation was deemed futile. In 57% of patients, the immunosuppressive therapy was not changed and only three patients were treated with chloroquine. Most patients recovered without experimental antiviral therapy. Modification of the immunosuppressive regimen alone could be a therapeutic option for SOT recipients suffering from moderate to severe COVID‐19. Pre‐existent frailty is associated with death from COVID‐19.