Dynamic outflow tract obstruction in congenitally corrected transposition of the great arteries

Andrew O Zurick1, Venu Menon

  • 1Heart & Vascular Institute, Department of Cardiovascular Medicine, Cleveland Clinic, Desk J1-5, Cleveland, OH 44195, USA. Zuricka@ccf.org

Insights

Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart defect. This case highlights severe complications and challenges in surgical treatment for adult CCTGA patients.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect, affecting 0.5% of cases.
  • Left ventricle outflow tract obstruction is a common complication in CCTGA, occurring in 44-57% of patients.

Observation:

  • A 45-year-old woman with CCTGA presented with worsening dyspnea and severe tricuspid regurgitation.
  • Imaging revealed severe right ventricle dysfunction and dynamic left ventricle outflow tract obstruction due to mitral valve systolic anterior motion and a ventricular septal aneurysm.

Findings:

  • The patient exhibited severe systemic ventricle (morphologic right ventricle) contractile dysfunction.
  • Dynamic non-systemic ventricle (morphologic left ventricle) outflow tract obstruction was identified, caused by systolic anterior motion of the mitral valve and a ventricular septal aneurysm.

Implications:

  • Surgical correction was deemed too high-risk for this patient.
  • Medical therapy was pursued as the primary treatment course for this complex adult CCTGA case.

Related Concept Videos

Aortic Regurgitation I: Introduction01:15

Aortic Regurgitation I: Introduction

IntroductionAortic regurgitation is characterized by the backward flow of blood from the aorta into the left ventricle during diastole and arises from the improper closure of the aortic valve. This condition results in left ventricular volume overload and can stem from both acute and chronic etiologies, each contributing uniquely to the disease's progression and symptomatology.Acute and Chronic CausesAcute aortic regurgitation often results from events that suddenly impair the integrity of the...
Pyloric Obstruction01:11

Pyloric Obstruction

Pyloric obstruction, also referred to as gastric outlet obstruction, is a condition characterized by narrowing or blockage at the pylorus—the muscular valve regulating the flow of stomach contents into the duodenum. When this passage becomes impaired, the stomach cannot effectively empty its contents into the small intestine. This disruption leads to a range of gastrointestinal symptoms, including early satiety, bloating, epigastric pain, postprandial nausea, persistent vomiting, and...
Trachea01:22

Trachea

The trachea, commonly known as the windpipe, is a vital part of the human respiratory system. It serves as a passageway for air to travel between the larynx and the bronchi, allowing oxygen to reach the lungs. Let's explore its anatomical features, dimensions, layers of the tracheal wall, associated muscles, and the functions of its parts.
Anatomical Features:
Location: About half of the trachea is situated in the neck, anterior to the esophagus, and extends from the larynx (at the level of the...
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...
Oxygen Delivering System II: Venturi Mask and Transtracheal Oxygen01:16

Oxygen Delivering System II: Venturi Mask and Transtracheal Oxygen

Oxygen therapy is a pivotal aspect of medical care, particularly for patients with respiratory ailments. Two prominent oxygen-delivering systems include the Venturi mask and the transtracheal oxygen catheter.
Venturi Mask
The Venturi mask, named after the Venturi effect, is designed to deliver precise oxygen concentrations. It consists of a large tube with an oxygen inlet that narrows down, causing a pressure drop that pulls air in through adjustable side ports. The mask is a lightweight,...
Intestinal Obstruction II: Pathophysiology01:07

Intestinal Obstruction II: Pathophysiology

Intestinal obstruction triggers a series of physiological responses, starting with gas and fluid accumulation in the bowel segment proximal to the obstruction, leading to distension. This distended intestine compresses the diaphragm, hindering lung expansion and potentially leading to reduced respiratory effort, atelectasis, and pneumonia.To overcome the blockage, the gut intensifies contractions, causing colicky abdominal pain, nausea, and vomiting, which reduces fluid and food intake and...