Persistent left vertical vein: An unusual cause of pulmonary hypertension and cirrhosis in a patient with hypertrophic obstructive cardiomyopathy.
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ABSTRACT:
SUBMITTER: Karabulut U
PROVIDER: S-EPMC8357436 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:AimsObstructive hypertrophic cardiomyopathy (oHCM) is characterized by dynamic obstruction of the left ventricular (LV) outflow tract (LVOT). Although this may be mediated by interplay between the hypertrophied septal wall, systolic anterior motion of the mitral valve, and papillary muscle abnormalities, the mechanistic role of LV shape is still not fully understood. This study sought to identify the LV end-diastolic morphology underpinning oHCM.Methods and resultsCardiovascular magnetic resonance images from 2398 HCM individuals were obtained as part of the NHLBI HCM Registry. Three-dimensional LV models were constructed and used, together with a principal component analysis, to build a statistical shape model capturing shape variations. A set of linear discriminant axes were built to define and quantify (Z-scores) the characteristic LV morphology associated with LVOT obstruction (LVOTO) under different physiological conditions and the relationship between LV phenotype and genotype. The LV remodelling pattern in oHCM consisted not only of basal septal hypertrophy but a combination with LV lengthening, apical dilatation, and LVOT inward remodelling. Salient differences were observed between obstructive cases at rest and stress. Genotype negative cases showed a tendency towards more obstructive phenotypes both at rest and stress.ConclusionsLV anatomy underpinning oHCM consists of basal septal hypertrophy, apical dilatation, LV lengthening, and LVOT inward remodelling. Differences between oHCM cases at rest and stress, as well as the relationship between LV phenotype and genotype, suggest different mechanisms for LVOTO. Proposed Z-scores render an opportunity of redefining management strategies based on the relationship between LV anatomy and LVOTO.
| S-EPMC10229266 | biostudies-literature
Project description:To understand the interplay between cardiomyocyte and nonmyocyte cell types in human obstructive and non-obstructive hypertrophic cardiomyopathy, single nuclei RNA-sequencing was performed on 2 unused donor hearts, 1 obstructive HCM specimen, and 6 non-obstructive HCM specimens.
2022-01-01 | GSE181764 | GEO
Project description:Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β-blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra-aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.
| S-EPMC8751867 | biostudies-literature
Project description: Not available
| S-EPMC5408548 | biostudies-literature
Project description:BackgroundHypertrophic cardiomyopathy (HCM), the most common genetic heart disease, is classified into hypertrophic non-obstructive and hypertrophic obstructive cardiomyopathy (HOCM). Patients with HOCM and coexisting heart failure or arterial hypertension are often prescribed afterload-reducing drugs. Although recommended in current guidelines, data on the direct effect of discontinuing afterload-reducing medication are scarce. This study aims to demonstrate the benefit of discontinuing afterload-reducing medication in HOCM patients.MethodsThis monocentric retrospective analysis included 24 patients with HOCM with afterload-reducing medication, including angiotensin-converting enzyme inhibitors, angiotensin-1 receptor blocker and dihydropyridine-calcium channel blocker, at their first outpatient visit. Effects of discontinuing this medication on LVOTO were examined compared to patients with persistent use despite medical advice.Results16 patients discontinued their afterload-reducing drugs, resulting in a significant decrease in median LVOT gradient from 86.5 [60.5-109.3] mmHg to 61.5 [28.3-97.50] mmHg (p = 0.0004). In 6 patients, beta-blocker therapy was initiated simultaneously, or the dose was increased. Regardless, LVOT gradient reduction was also significant in the remaining 10 patients (p = 0.001). The gradient was not changed significantly in the 8 patients continuing their afterload-reducing medication.ConclusionsDiscontinuation of afterload-reducing drugs significantly decreases LVOTO. Our study underscores the significance of abstaining from afterload-reducing drugs in HOCM patients, particularly in patients with concomitant hypertension or heart failure. According to recently published European guidelines, HOCM patients should preferably be treated with beta-blockers or non-dihydropyridine-calcium channel blockers.
| S-EPMC11286422 | biostudies-literature
Project description:In obstructive hypertrophic cardiomyopathy patients with preserved left ventricular (LV) ejection fraction, we sought to determine whether LV global longitudinal strain (LV-GLS) provided incremental prognostic utility.We studied 1019 patients with documented hypertrophic cardiomyopathy (mean age, 50±12 years; 63% men) evaluated at our center between 2001 and 2011. We excluded age <18 years, maximal LV outflow tract gradient <30 mm Hg, bundle branch block or atrial fibrillation, past pacemaker/cardiac surgery, including myectomy/alcohol ablation, and obstructive coronary artery disease. Average resting LV-GLS was measured offline on 2-, 3-, 4-chamber views using Velocity Vector Imaging (Siemens, Malvern, PA). Outcome was a composite of cardiac death and appropriate internal defibrillator (implantable cardioverter defibrillator) discharge. Maximal LV thickness, LV ejection fraction, indexed left atrial dimension, rest and maximal LV outflow tract gradient, and LV-GLS were 2.0±0.2 cm, 62±4%, 2.2±4 cm/m2, 52±42 mm Hg, 103±36 mm Hg, and -13.6±4%. During 9.4±3 years of follow-up, 668 (66%), 166 (16%), and 122 (20%), respectively, had myectomy, atrial fibrillation, and implantable cardioverter defibrillator implantation, whereas 69 (7%) had composite events (62 cardiac deaths). Multivariable competing risk regression analysis revealed that higher age (subhazard ratio, 1.04 [1.02-1.07]), AF during follow-up (subhazard ratio, 1.39 [1.11-1.69]), and worsening LV-GLS (subhazard ratio, 1.11 [1.05-1.22]) were associated with worse outcomes, whereas myectomy (subhazard ratio, 0.44 [0.25-0.72]) was associated with improved outcomes (all P<0.01). Sixty-one percent of events occurred in patients with LV-GLS worse than median (-13.7%).In obstructive hypertrophic cardiomyopathy patients with preserved LV ejection fraction, abnormal LV-GLS was independently associated with higher events, whereas myectomy was associated with improved outcomes.
| S-EPMC5721859 | biostudies-other
Project description:AimsNot all obstructive hypertrophic cardiomyopathy (HCM) patients are symptomatic. The relation between obstructive HCM and symptoms is not well understood. The hypothesis of this study is that left-ventricular outflow tract (LVOT) acceleration time (AT) is associated with symptoms.MethodsWe included 187 patients (61% men, mean age 55 ± 14 years) with obstructive HCM, defined as a maximal wall thickness ≥ 15 mm and a resting or provoked LVOT peak gradient ≥ 30 mmHg. Peak velocity (PV), left-ventricular (LV) ejection time (ET), and AT (the time between LVOT flow onset and the moment of PV) were measured on continuous-wave (CW) Doppler tracings. Logistic and Cox proportional hazard regression analyses were used to evaluate the relation between symptoms [New York Heart Association (NYHA) class ≥ II] and echocardiographic measurements, including AT. Reproducibility was assessed using the intraclass correlation coefficient (ICC).ResultsSymptomatic patients were more often female and had higher mean AT values. Logistic regression demonstrated a significant association between AT and symptomatic status (odds ratio 1.31 per 10 ms, p < 0.01) after adjustment for sex, negative inotropes, PV, LVOT diameter, and diastolic dysfunction. AT was independently associated with symptoms and septal reduction during follow-up (hazard ratio 1.09 per 10 ms, p < 0.05). The ICC was 0.98 with a mean difference of 0.28 ± 8.4 ms.ConclusionIn obstructive HCM patients, increased AT is significantly related to symptoms after adjustment for sex, negative inotropes, PV, LVOT diameter, and diastolic dysfunction, and is associated with the symptomatic status during follow-up. AT represents an easily measured echocardiographic variable with excellent inter-reader reproducibility.
| S-EPMC8363723 | biostudies-literature
Project description:The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is an uncommon cause of cardiac hypertrophy, fibrosis, and dysfunction. It shares similar features to numerous other causes of left ventricular hypertrophy, and therefore, because of its rarity, may not be immediately considered as a diagnosis. Prompt recognition of clinical and cardiac imaging features may expedite diagnosis and management. We report the case of a 38-year-old man admitted with neurological symptoms and in whom the diagnostic workup led to the diagnosis of MELAS syndrome with cardiac involvement.
| S-EPMC7917926 | biostudies-literature
Project description:Surgical septal myectomy is the treatment of choice for patients with symptomatic hypertrophic obstructive cardiomyopathy refractory to medications. This report describes our minimally invasive approach for performing a septal myectomy via a ministernotomy that has been used at our institution for more than a decade. In particular, patient preparation, surgical technique, and clinical considerations are highlighted. Performed properly, this minimally invasive technique is a feasible and effective approach in our experience.
| S-EPMC5774662 | biostudies-literature
Project description:This case report concerns an 81-year-old woman with previously well-controlled hypertrophic obstructive cardiomyopathy (HOCM). She was referred to our hospital because of the acute onset of takotsubo syndrome. Echocardiography revealed basal hyperkinesis due to takotsubo syndrome superimposed on septal hypertrophy, which resulted in the reappearance of prominent left ventricular outflow tract obstruction (LVOTO). Although she developed cardiogenic shock triggered by atrial fibrillation, LVOTO was successfully mitigated by aggressive fluid resuscitation, rhythm control, and the administration of β-blocker. We herein report a rare case with catastrophic hemodynamics due to the incidental combination of HOCM and takotsubo syndrome.
| S-EPMC7184076 | biostudies-literature