Isolated Left Ventricular Apical Hypoplasia.
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ABSTRACT:
SUBMITTER: Dattani A
PROVIDER: S-EPMC8728883 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
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Isolated Left Ventricular Apical Hypoplasia.
Dattani Abhishek A Prasad Rachana R
Cardiac failure review 20210301
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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
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
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
Project description:Isolated left ventricular hypoplasia is an entity with uncertain natural history and etiology. The presentation could vary from being asymptomatic to sudden death. This form of cardiomyopathy has been reported in infants as well as in adults. This case report aims to alert physicians to this diagnosis and the undeniable advantage of cardiac magnetic resonance.
| S-EPMC7727894 | biostudies-literature
Project description:We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.
| S-EPMC4720816 | biostudies-literature
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
Project description: Not available
| S-EPMC1994450 | biostudies-literature
Project description:Left ventricular apical thrombus is a known complication following an anterior ST-elevation myocardial infarction. Although left ventriculography may suggest an apical thrombus in the presence of a filling defect, additional imaging with echocardiography and/or cardiac magnetic resonance is strongly recommended to further characterize the thrombus post myocardial infarction.
| S-EPMC6452471 | biostudies-literature
Project description: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.>.
| S-EPMC8758584 | biostudies-literature
Project description:Penetrating injuries of the thorax and abdomen, such as gunshot and stabbing, are rare in children. We present the case of a pediatric patient with a history of remote gunshot injury presenting with a late aneurysm in the left ventricle. (Level of Difficulty: Intermediate.).
| S-EPMC8305065 | biostudies-literature