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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

150
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...
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Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

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In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.3K
Acute Respiratory Failure-II01:21

Acute Respiratory Failure-II

171
Type I Respiratory Failure, or hypoxemic respiratory failure, occurs when the partial pressure of oxygen (PaO2) in arterial blood falls below 60 mmHg while breathing room air without a corresponding increase in arterial carbon dioxide levels (PaCO2). This condition highlights a significant impairment in the lungs' capacity to oxygenate the blood.
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
171
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

171
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,...
171
Other Pulmonary Disorders01:17

Other Pulmonary Disorders

801
Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
801
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

135
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...
135

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Isolation of Pulmonary Artery Smooth Muscle Cells from Neonatal Mice
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Pulmonary hypertension in infancy: a reversible cause.

Cassandra Campbell1, Kawaljeet Singh2, Mark Weems2

  • 1Pediatrics, The University of Tennessee Health Science Center, Memphis, Tennessee, USA cassie.devol@gmail.com.

BMJ Case Reports
|January 3, 2025
PubMed
Summary

A rare congenital heart defect, supravalvular mitral ring, caused severe pulmonary hypertension in an infant. Surgical removal of the membrane successfully treated the condition, highlighting the importance of accurate diagnosis.

Keywords:
Congenital disordersPulmonary hypertensionValvar diseases

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Pulmonary Hypertension

Background:

  • Pulmonary hypertension (PH) in infants often stems from conditions like bronchopulmonary dysplasia.
  • Accurate diagnosis is crucial as some causes of PH are reversible.

Observation:

  • A healthy infant presented with respiratory distress and severe PH of unclear origin.
  • Pulmonary vasodilators were administered to reduce right ventricular afterload.
  • Imaging identified a supravalvular mitral ring as the underlying cause of PH.

Findings:

  • Surgical resection of the supravalvular mitral ring membrane resolved the PH.
  • Supravalvular mitral ring is a rare left-sided congenital heart lesion.
  • Concurrent atrial septal defects can mask the left ventricular inflow gradient, complicating diagnosis.

Implications:

  • Early and accurate diagnosis of supravalvular mitral ring is vital for appropriate treatment.
  • Misdiagnosis can lead to inappropriate use of pulmonary vasodilators, potentially worsening the infant's condition.
  • This case underscores the importance of considering rare congenital heart defects in the differential diagnosis of infant PH.