Browse
Submit Data
Databases
API
Help

Dataset Information

24 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Progression of left ventricular apical hypoplasia-like restrictive cardiomyopathy with severe pulmonary hypertension: Follow-up from fetal stage.

ABSTRACT: Left ventricular (LV) apical hypoplasia is a rare restrictive cardiomyopathy subtype with an unclear pathophysiology. LV apical hypoplasia typically presents with elongated right ventricle (RV) wrapping around a truncated and spherical LV with a deficient apex (the "banana-shape" of the RV). Here we report a case of a young boy with apparent LV apical hypoplasia that developed after birth; no "banana-shaped" RV was observed during the fetal period. Moreover, suprasystemic pulmonary hypertension (PH) developed even after a mitral valve replacement was performed for progressive mitral stenosis and regurgitation at 14 months of age. He underwent surgery for the Potts shunt, a shunt between the pulmonary artery and aorta, at 13 years to secure systemic output. His PH ameliorated and his heart failure remained stable for 3 years after the operation. This case indicates that the "banana-shaped" RV seen in this condition is not always congenital but that it can form and develop after birth. Furthermore, this case supports the usefulness of the Potts shunt as a therapeutic option in patients with severe PH due to LV apical hypoplasia. <Learning objective: Left ventricular apical hypoplasia typically presents with elongated right ventricle wrapping around a truncated and spherical left ventricle with a deficient apex. However, this characteristic may not be always congenital and can also form and develop after birth. The Potts shunt, a shunt between the pulmonary artery and aorta, may be a therapeutic option in patients with severe pulmonary hypertension due to left ventricular apical hypoplasia.>.

SUBMITTER: Sato R

PROVIDER: S-EPMC8758584 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Similar Datasets

Isolated Left Ventricular Apical Hypoplasia.

Project description: Not available

| S-EPMC8728883 | biostudies-literature

Isolated left ventricular apical hypoplasia: case report.

Project description:BackgroundIsolated left ventricular apical hypoplasia (ILVAH) is a rare, probably congenital, cardiac condition recently reported in the last two decades. Although most cases are asymptomatic or mildly symptomatic, some severe and fatal cases have been reported, leading to more efforts for appropriate diagnosis and treatment. We describe the first, and severe, case of this pathology in Peru and Latin America.Case summaryA 24-year-old male with a history of long-standing alcohol and illicit drug use presented with symptoms of heart failure (HF) and atrial fibrillation (AF). Transthoracic echocardiography showed biventricular dysfunction along with spherical left ventricle, abnormal papillary muscles' origin from the left ventricular apex, and an elongated right ventricle wrapping around the deficient left ventricular apex. Cardiac magnetic resonance confirmed these findings and revealed subepicardial fatty replacement at the left ventricular apex. The diagnosis of ILVAH was made. He was discharged from hospital with carvedilol, enalapril, digoxin, and warfarin. Eighteen months later he remains mildly symptomatic at New York Heart Association functional class II without worsening HF nor thrombo-embolism.DiscussionThis case highlights the usefulness of multimodality non-invasive cardiovascular imaging for accurate diagnosis of ILVAH as well as the importance of close follow-up and treatment of established complications (HF and AF).

| S-EPMC9945848 | biostudies-literature

Cardiac magnetic resonance imaging findings of isolated left ventricular apical hypoplasia.

Project description:Isolated left ventricular apical hypoplasia (ILVAH) is an uncommon and likely congenital cardiac abnormality that has been described as relatively new. ILVAH is characterized by a truncated, globular-shaped left ventricle (LV) with bulging of the interventricular septum toward the right ventricle (RV), wrapping of an elongated and lengthened RV around the absent LV apex, thinning and fat replacement of apical myocardium of the LV, and abnormalities in the papillary muscle arrangement of the LV. In this report, we present the cardiac magnetic resonance imaging findings of a 22-year-old female patient with non-specific cardiac complaints that were compatible with ILVAH. Recognition of this rare cardiomyopathy is important for clinicians and radiologists in order to follow up on patients with ILVAH, as it may lead to severe complications, and to distinguish it from other cardiomyopathies.Learning objectiveIsolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiomyopathy that has some serious complications, such as left-sided heart failure, severe pulmonary hypertension, and fatal arrhythmias. By recognizing and identifying the cardiac magnetic resonance imaging findings of ILVAH, clinicians and radiologists can take appropriate measures to manage and treat patients with this condition, potentially improving outcomes and reducing the risk of complications.

| S-EPMC10658309 | biostudies-literature

Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report.

Project description:BackgroundIsolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac abnormality, which might result in severe symptomatic heart failure (HF) with pulmonary hypertension, atrial fibrillation (AF), or malignant ventricular tachycardia in adults.Case summaryA 32-years-old man presented with exertional dyspnoea New York Heart Association Class II and persistent AF. Echocardiography and cardiac magnetic resonance showed the presence of (i) spherical remodelling of the left ventricle (LV) with impaired contractile function (three-dimensional ejection fraction, EF 32%); (ii) substitution of apical myocardium by fatty tissue; (iii) abnormal origin of a papillary muscle network; and (iv) an elongated right ventricle, compatible with ILVAH. In addition, non-compacted endomyocardial layer of the LV was observed. Because of a high risk of sudden cardiac death in symptomatic HF patients with reduced EF, an implantable cardioverter-defibrillator was placed which followed by pulmonary vein isolation. After the procedures and restoration of sinus rhythm, the patient demonstrated improvement in HF symptoms and exercise tolerance. This was accompanied by an enhancement of left and right ventricular systolic function by echocardiography. At 6-month, 1, and 2-year follow-up the clinical conditions of the patient and echocardiographic findings remained stable.DiscussionA rare combination of ILVAH and left ventricular myocardium non-compaction was observed in this young adult who presented with symptomatic HF and persistent AF. The use of consecutive invasive cardiac procedures leads to restoration of sinus rhythm, the improvement of myocardial contractility and clinical manifestation of HF.

| S-EPMC7047060 | biostudies-literature

Calcified aneurysm of left ventricular apex in apical cardiomyopathy.

Project description: Not available

| S-EPMC3461688 | biostudies-literature

Left ventricular apical aneurysm in chronic Chagas cardiomyopathy-A case report.

Project description:A 56-year-old female, who immigrated to the USA from Honduras, presented with worsening shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, and decreased exercise tolerance over the previous 2 months. She was diagnosed 1 year previously with non-ischemic dilated cardiomyopathy and non-sustained monomorphic ventricular tachycardia. An implantable cardioverter defibrillator was placed. Cause for her dilated cardiomyopathy was unknown at that time. On admission, her electrocardiogram showed low voltage complexes, with frequent premature ventricular contractions. Transthoracic two-dimensional echocardiogram (2D ECHO) showed severely reduced ejection fraction of 20%, severe mitral regurgitation with left ventricular (LV) hypokinesis, and inferolateral and inferior wall akinesis. On review of her records, a contrast 2D ECHO from the previous year revealed an aneurysm of the LV apical region. Live three-dimensional (3D) ECHO on her present admission showed persistent LV apical aneurysm. Computed tomography angiogram showed no atherosclerotic lesions. Multiple episodes of non-sustained ventricular tachycardia were recorded on telemetry. Based on these findings, the diagnosis of Chagas cardiomyopathy was entertained. Serological tests for Trypanosoma cruzi antibodies were done and returned positive. We report a case of chronic Chagas cardiomyopathy that was initially missed but ultimately diagnosed based on the finding of LV apical aneurysm. <Learning objective: Chagas cardiomyopathy is becoming increasingly prevalent in non-endemic countries. One hallmark finding associated with Chagas cardiomyopathy is left ventricle apical aneurysm. We were able to identify this finding with transthoracic contrast 2D ECHO and live 3D ECHO.>.

| S-EPMC6281735 | biostudies-literature

Induction of left ventricular hypoplasia by occluding the foramen ovale in the fetal lamb.

Project description:Disturbed fetal haemodynamics often affects cardiac development and leads to congenital cardiac defects. Reduced left ventricular (LV) preload in the fetus may result in hypoplastic LV, mitral and aortic valve, mimicking a moderate form of hypoplastic left heart complex. We aimed to induce LV hypoplasia by occluding the foramen ovale (FO) to reduce LV preload in the fetal sheep heart, using percutaneous trans-hepatic catheterisation. Under maternal anaesthesia and ultrasound guidance, hepatic venous puncture was performed in six fetal lambs (0.7-0.75 gestation). A coronary guidewire was advanced into the fetal inferior vena cava, right and left atrium. A self-expandable stent was positioned across the FO. An Amplatzer Duct Occluder was anchored within the stent for FO occlusion. Euthanasia and post-mortem examination was performed after 3 weeks. Nine fetuses were used as age-matched controls. Morphometric measurements and cardiac histopathology were performed. Compared with controls, fetal hearts with occluded FO had smaller LV chamber, smaller mitral and aortic valves, lower LV-to-RV ratio in ventricular weight and wall volume, and lower number of LV cardiomyocyte nuclei. We conclude that fetal FO occlusion leads to a phenotype simulating LV hypoplasia. This large animal model may be useful for understanding and devising therapies for LV hypoplasia.

| S-EPMC6972793 | biostudies-literature

Left ventricular apical diseases.

Project description:There are many disorders that may involve the left ventricular (LV) apex; however, they are sometimes difficult to differentiate. In this setting cardiac imaging methods can provide the clue to obtaining the diagnosis. The purpose of this review is to illustrate the spectrum of diseases that most frequently affect the apex of the LV including Tako-Tsubo cardiomyopathy, LV aneurysms and pseudoaneurysms, apical diverticula, apical ventricular remodelling, apical hypertrophic cardiomyopathy, LV non-compaction, arrhythmogenic right ventricular dysplasia with LV involvement and LV false tendons, with an emphasis on the diagnostic criteria and imaging features. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s13244-011-0091-6) contains supplementary material, which is available to authorized users.

| S-EPMC3259385 | biostudies-literature

Diagnosis of apical hypertrophic cardiomyopathy: T-wave inversion and relative but not absolute apical left ventricular hypertrophy.

Project description:BackgroundDiagnosis of apical HCM utilizes conventional wall thickness criteria. The normal left ventricular wall thins towards the apex such that normal values are lower in the apical versus the basal segments. The impact of this on the diagnosis of apical hypertrophic cardiomyopathy has not been evaluated.MethodsWe performed a retrospective review of 2662 consecutive CMR referrals, of which 75 patients were identified in whom there was abnormal T-wave inversion on ECG and a clinical suspicion of hypertrophic cardiomyopathy. These were retrospectively analyzed for imaging features consistent with cardiomyopathy, specifically: relative apical hypertrophy, left atrial dilatation, scar, apical cavity obliteration or apical aneurysm. For comparison, the same evaluation was performed in 60 healthy volunteers and 50 hypertensive patients.ResultsOf the 75 patients, 48 met conventional HCM diagnostic criteria and went on to act as another comparator group. Twenty-seven did not meet criteria for HCM and of these 5 had no relative apical hypertrophy and were not analyzed further. The remaining 22 patients had relative apical thickening with an apical:basal wall thickness ratio >1 and a higher prevalence of features consistent with a cardiomyopathy than in the control groups with 54% having 2 or more of the 4 features. No individual in the healthy volunteer group had more than one feature and no hypertension patient had more than 2.ConclusionA cohort of individuals exist with T wave inversion, relative apical hypertrophy and additional imaging features of HCM suggesting an apical HCM phenotype not captured by existing diagnostic criteria.

| S-EPMC4392393 | biostudies-literature

Apical hypertrophic cardiomyopathy with a plexus of coronary artery to left ventricular fistulae.

Project description: Not available

| S-EPMC8343557 | biostudies-literature