Left Ventricular Hypertrophy and Hypertrophic Cardiomyopathy in Adult Solid Organ Transplant Recipients

Abstract

Background. Hypertrophic cardiomyopathy (HCM) in pediatric solid organ transplant recipients has been reported in association with use of calcineurin inhibitors. However, data on the incidence and prevalence of HCM in adult posttransplant patients are limited. We sought to describe the clinical characteristics of solid organ transplant recipients who were diagnosed with HCM from 2011 to 2021 at a single center. Methods. Patients who had undergone solid organ transplant and exhibited left ventricular hypertrophy with left ventricular wall thickness ≥13 mm on transthoracic echocardiography were included. Clinical history, pedigree analysis, clinical genetic testing, transthoracic echocardiography, cardiac magnetic resonance imaging, treatment, and follow-up testing results were collected. Categorical variables were described as n (%). Continuous variables were described with medians and interquartile ranges and compared using the Wilcoxon rank-sum and Kruskal-Wallis tests. A 2-sided P < 0.05 was considered statistically significant. Results. Three lung, 5 kidney, and 4 liver transplant recipients from 12 different families were included. Seven patients (58%) did not carry a preexisting diagnosis of hypertension, and none had a history of aortic or subaortic stenosis. A majority of patients exhibited asymmetric septal hypertrophy (67%; medial septal thickness versus left ventricular posterior wall thickness 17 versus 13 mm; P < 0.001) and dynamic left ventricular outflow tract (LVOT) obstruction (58%). All patients were managed long term with calcineurin inhibitors. Clinical genetic testing in 6 patients identified 2 with disease-causing variants in 2 sarcomere genes, myosin binding protein-C and myosin heavy chain 7. Four patients (33%) underwent successful septal reduction therapy for treatment of symptomatic LVOT obstruction. Conclusions. Symptomatic HCM with dynamic LVOT obstruction can develop in solid organ transplant recipients, and genetic testing can identify individuals with sarcomeric HCM. Medical management and septal reduction therapies are treatment options for severe symptomatic disease.

Mitochondrial dysfunction in the pathogenesis of hypertrophic cardiomyopathy

“…Утолщение миокарда при ГКМП снижает способность левого желудочка расслабляться и наполняться кровью во время диастолы [5]. В некоторых случаях ГКМП утолщённая межжелудочковая перегородка может препятствовать оттоку крови из левого желудочка, что приводит к обструкции его выносящего тракта и обструктивной ГКМП [6]. Патоморфогенез ГКМП представляет собой многофакторный процесс, включающий сложное взаимодействие генетических, клеточных и тканевых изменений, которые приводят к гипертрофии миокарда и сопутствующим метаболическим нарушениям, обусловливающим клинические проявления, такие как сердечная недостаточность, нарушения ритма сердца, а также внезапная смерть [7].…”

Differences in Cardiovascular Remodeling in Kidney Transplant Recipients and Peritoneal Dialysis Patients

“…At the beginning of dialysis, 75% of patients with endstage renal disease (ESRD) have LVH, primarily because of hypertension, volume expansion, and anaemia. Kidney transplantation is the optimal treatment for ESRD and is associated with reduced cardiovascular morbidity and improved quality of life and survival [5]. Some echocardiographic studies have reported reduced left ventricular mass and improved left ventricular ejection fraction (LVEF), but these findings have been inconsistent.…”

“…In fact, serial cardiac magnetic resonance imaging has failed to detect a significant reduction in left ventricular mass following kidney transplantation. In a number of studies, successful kidney transplantation has been associated with significant echocardiographic regression of LVH in KTx (kidney transplant) [5,6], while other studies have shown no positive effect. The expansion and variation of intravascular volume in dialysis patients compared with KTx have been suggested as one of the reasons for the contradictory result [7,8].…”