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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

644
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
644
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

601
Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
601
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

467
Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
467
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

508
Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
508
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

619
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
619
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

532
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
532

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Related Experiment Video

Updated: Feb 1, 2026

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
10:03

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

783

Gerbode defect misinterpreted as pulmonary hypertension.

Ruchira Garg1, Richard Garcia1, Robert J Cubeddu2

  • 1Miami Children's Hospital, 3100 S.W. 62nd Avenue, Miami, FL 33155-3009, USA.

Journal of Cardiology Cases
|December 12, 2018
PubMed
Summary
This summary is machine-generated.

A rare Gerbode defect, a left ventricle-to-right atrium shunt, can be misdiagnosed as pulmonary hypertension in adult congenital heart disease patients. Noninvasive imaging can confirm this diagnosis with high clinical suspicion.

Keywords:
Congenital heart diseaseEchocardiographyPulmonary hypertensionShuntVentricular septal defect

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Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
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Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
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Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease
  • Medical Imaging

Background:

  • Adult congenital heart disease (CHD) management presents complex challenges for cardiologists.
  • Patients with complex CHD and prior surgeries require specialized care.
  • Misdiagnosis of cardiac conditions can occur in adult CHD patients.

Purpose of the Study:

  • To highlight the diagnostic challenges of a Gerbode defect in adult CHD.
  • To present a case of misdiagnosed pulmonary hypertension due to a Gerbode defect.
  • To emphasize the utility of noninvasive imaging in diagnosing Gerbode defects.

Main Methods:

  • Case report of a 64-year-old woman with a history of CHD and multiple cardiac surgeries.
  • Initial diagnosis via transthoracic echocardiogram suggested pulmonary hypertension and VSD.
  • Comprehensive noninvasive evaluation including cardiac MRI confirmed a Gerbode defect.

Main Results:

  • A Gerbode defect (left ventricle-to-right atrium shunt) was identified, not severe pulmonary hypertension.
  • The high-velocity jet was misinterpreted as tricuspid regurgitation.
  • The ventricular septal defect was found to be small and restrictive with normal pulmonary pressures.

Conclusions:

  • Gerbode defects can be challenging to diagnose in adult CHD patients.
  • Noninvasive imaging strategies, particularly echocardiography and cardiac MRI, are crucial for diagnosis.
  • High clinical suspicion combined with advanced imaging can accurately identify Gerbode defects, preventing misdiagnosis.