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Related Concept Videos

Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
Heart Failure II: Pathophysiology01:29

Heart Failure II: Pathophysiology

Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
Heart Failure III: Clinical Manifestations01:26

Heart Failure III: Clinical Manifestations

Heart failure (HF) manifests primarily as dyspnea, fatigue, and fluid retention, resulting in peripheral and pulmonary edema. Symptoms may vary depending on which ventricle is more affected, left or right.Left-Sided Heart FailureAlso known as left ventricular failure, this condition results from the left ventricle's inability to fill or eject sufficient blood into the systemic circulation. It leads to pulmonary congestion, which occurs when the left ventricle fails to eject blood effectively...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...

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Implantation of Total Artificial Heart in Congenital Heart Disease
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Case Report--Late Presentation of Shone's Complex With Severe Left Ventricular Dysfunction.

Refal Mahrouqi1, Asayil Almarjan1, Ahmed Sultan2,3

  • 1Faculty of Medicine, Umm Al-Qura University, Makkah, Kingdom of Saudi Arabia.

Journal of the Saudi Heart Association
|April 16, 2026
PubMed
Summary

This case highlights a complex congenital heart disease (CHD) presentation in a young patient. Surgical intervention successfully addressed multiple cardiac anomalies, improving the patient's condition.

Keywords:
Congenital heart diseaseLeft ventricular outflow tract obstructionParachute mitral valveShone’s complex

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Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Shone's complex is a rare congenital heart disease (CHD) with variable anatomical and clinical features.
  • It commonly involves supravalvular mitral ring, parachute Mitral Valve (MV), subaortic stenosis, and coarctation of the aorta.

Purpose of the Study:

  • To present a complex case of Shone's complex in a 14-year-old girl with severe cardiac failure.
  • To detail the diagnostic findings and surgical management of this rare CHD variant.

Main Methods:

  • Diagnostic evaluation included echocardiography and CT.
  • Surgical intervention involved Mitral Valve (MV) replacement, Konno procedure with Left Ventricular Outflow Tract (LVOT) and annular enlargement, Aortic Valve (AV) replacement, and Tricuspid Valve (TV) repair.

Main Results:

  • The patient presented with severe cachexia, cardiac failure, bicuspid Aortic Valve (AV), severe valvular and subvalvular aortic stenosis, subaortic membrane, LVOT obstruction, dilated Left Ventricle (LV) with reduced ejection fraction (EF), and a ventricular septal defect.
  • The patient experienced an uneventful intensive care unit (ICU) course post-operatively.

Conclusions:

  • Complex congenital heart disease, such as Shone's complex, requires comprehensive diagnostic assessment.
  • Multistage surgical repair can effectively manage severe forms of Shone's complex, leading to favorable outcomes.