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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Mitral regurgitation is characterized by the backward circulation of blood from the left ventricle to the left atrium during systole, a phase of the cardiac cycle when the heart contracts and pumps blood out of the chambers. This abnormal flow occurs primarily due to the dysfunction of the mitral valve or its supporting structures, which include the mitral leaflets, chordae tendineae, annulus, and papillary muscles.Etiology and Mechanisms:Primary Mitral Regurgitation: This type arises from...
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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Cortriatriatum with classical Raghib complex: a rare anatomic association.

Vinitha Viswambharan Nair1, Palleti Rajashekar, Anita Saxena

  • 1Department of CTVS, Cardio thoracic centre, All India Institute of Medical Sciences, New Delhi, India.

World Journal for Pediatric & Congenital Heart Surgery
|March 27, 2014
PubMed
Summary

Congenital heart defect cor triatriatum sinistrum with Raghib's complex is rare. Surgical correction successfully treated an infant with this complex condition, improving respiratory symptoms.

Keywords:
Raghib complexcortriatriatumcyanosisleft superior venacava

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Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Congenital Heart Disease

Background:

  • Cor triatriatum sinistrum is a rare congenital heart anomaly where the left atrium is divided into two chambers by a membrane.
  • Raghib's complex, involving a persistent left superior vena cava draining into the left atrium via an unroofed coronary sinus, is an infrequent co-occurrence.

Observation:

  • An 18-month-old infant presented with tachypnea and cyanosis, indicative of significant cardiopulmonary compromise.
  • Diagnostic imaging revealed cor triatriatum sinistrum and a persistent left superior vena cava draining abnormally into the left atrium.

Findings:

  • The infant was diagnosed with a rare combination of cor triatriatum sinistrum and Raghib's complex.
  • The persistent left superior vena cava drained into the left atrium through an unroofed coronary sinus, exacerbating the cardiac defect.

Implications:

  • Successful surgical intervention involved excising the cor triatriatum membrane and redirecting the persistent left superior vena cava to the right atrium.
  • This case highlights the importance of recognizing and surgically managing this rare complex congenital heart defect in infants.