Related Experiment Video

Updated: Feb 15, 2026

Author Spotlight: Oral Candida Diagnosis to Advance Clinical Treatment Regimen for pSS Patients
05:26

Author Spotlight: Oral Candida Diagnosis to Advance Clinical Treatment Regimen for pSS Patients

Published on: March 1, 2024

2.1K

Double-chambered right ventricle in a 16-year-old patient with Williams syndrome

Wojciech Mądry1, Maciej A Karolczak1, Ewa Zacharska-Kokot1

  • 1Department of Pediatric Cardiothoracic Surgery, Medical University of Warsaw, Warsaw, Poland.

Journal of Ultrasonography
|January 30, 2018
PubMed

Insights

A rare case of double-chambered right ventricle in a Williams syndrome patient was diagnosed via echocardiography. Surgical correction was successful, highlighting the importance of advanced imaging in complex congenital heart defects.

Area of Science:

  • Cardiology
  • Medical Genetics

Background:

  • Williams syndrome is a genetic disorder associated with various cardiovascular anomalies.
  • Double-chambered right ventricle (DCRV) is a rare congenital heart defect characterized by anomalous muscular septation of the right ventricle.
  • The co-occurrence of DCRV and Williams syndrome is exceptionally uncommon.

Observation:

  • A 16-year-old male with Williams syndrome presented with gastrointestinal bleeding, necessitating colonoscopy preparation.
  • Pre-existing diagnoses included ventricular septal defect and mild pulmonary stenosis.
  • Echocardiography under general anesthesia revealed hypertrophied muscular bundles causing severe right ventricular outflow tract obstruction with a 100 mmHg gradient.

Findings:

  • The patient was diagnosed with double-chambered right ventricle, a condition rarely reported in individuals with Williams syndrome.
  • Previous literature documented only one case of DCRV in Williams syndrome, diagnosed via invasive angiocardiography.
  • Echocardiography provided crucial data for diagnosis and surgical planning.

Implications:

  • This case underscores the importance of comprehensive cardiac evaluation in patients with Williams syndrome, even with seemingly unrelated presenting symptoms.
  • Echocardiography is a valuable non-invasive tool for diagnosing complex congenital heart defects like DCRV in this population.
  • Successful surgical correction based on echocardiographic findings demonstrates the efficacy of modern diagnostic and therapeutic approaches for rare cardiac anomalies.

Keywords:
Williams syndromedouble-chambered right ventricleechocardiography

More Related Videos

Assessment of Respiratory Function in Conscious Mice by Double-chamber Plethysmography08:58

Assessment of Respiratory Function in Conscious Mice by Double-chamber Plethysmography

Published on: July 10, 2018

44.1K
Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells08:52

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells

Published on: June 13, 2018

9.3K

Related Concept Videos

Cattell's 16 Personality Factors01:24

Cattell's 16 Personality Factors

Raymond Cattell's trait theory offers a structured framework for understanding personality by distinguishing between two critical traits: surface and source traits. Surface traits are observable patterns of behavior, such as indecisiveness, anxiety, and irrational fears. These traits are less stable, varying across situations and over time. This means that they are less helpful in understanding the deeper aspects of an individual's personality.
In contrast, source traits are the...
2.6K
Anatomy of the Brain: Ventricles01:18

Anatomy of the Brain: Ventricles

There are hollow fluid-filled cavities known as ventricles deep inside the human brain. There are two lateral ventricles, one in each cerebral hemisphere, and each has three different projections — the anterior, inferior, and posterior horns visible from the lateral side. A thin membrane called the septum pellucidum separates the two lateral ventricles. The slender third ventricle in the diencephalon is connected to each lateral ventricle via a channel called the interventricular foramen.
9.3K
Chambers of the Heart01:16

Chambers of the Heart

The human heart is a complex organ made up of four chambers: the right and left atria and the right and left ventricles. These internal chambers are separated by partitions known as the interatrial and interventricular septa. The exterior of the heart features a groove known as the coronary sulcus that demarcates the atria from the ventricles, while the anterior and posterior interventricular sulci distinguish between the two ventricles.
Deoxygenated blood from the body is received in the right...
10.7K
Fixing Double-strand Breaks02:04

Fixing Double-strand Breaks

The double-stranded structure of DNA has two major advantages. First, it serves as a safe repository of genetic information where one strand serves as the back-up in case the other strand is damaged. Second, the double-helical structure can be wrapped around proteins called histones to form nucleosomes, which can then be tightly wound to form chromosomes. This way, DNA chains up to 2 inches long can be contained within microscopic structures in a cell. A double-stranded break not only damages...
15.0K
Fixing Double-strand Breaks02:04

Fixing Double-strand Breaks

4.5K
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
701