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Updated: Feb 26, 2026

Comprehensive Echocardiographic Assessment of Right Ventricle Function in a Rat Model of Pulmonary Arterial Hypertension

Comprehensive Echocardiographic Assessment of Right Ventricle Function in a Rat Model of Pulmonary Arterial Hypertension

Published on: January 20, 2023

閉塞性肥大型心筋症におけるマバカムテン投与後の逆説的肺うっ血：血行動態遷移を例証する症例報告

Noriaki Tabata¹, Yasuhiro Izumiya², Noriko Nomura³

¹Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; Tabata Internal Medicine and Heart Clinic, Kumamoto, Japan.

JACC. Case reports

|February 24, 2026

要旨

Background:

Mavacamten effectively reduces left ventricular outflow tract (LVOT) obstruction in hypertrophic cardiomyopathy (HCM).

Case Summary:

A 51-year-old man with obstructive HCM developed paradoxical pulmonary congestion after successful LVOT relief with mavacamten. Despite a dramatic reduction in LVOT gradient and preserved ejection fraction, he experienced dyspnea and pleural effusion in week 6. Notably, an increase in tricuspid regurgitation pressure gradient and E/A ratio preceded clinical congestion. Initiation of an SGLT2 inhibitor led to clinical stabilization and hemodynamic re-equilibration by week 8.

Discussion:

This case illustrates a "functional dam" mechanism, where rapid LVOT obstruction relief may increase forward flow, potentially overwhelming a noncompliant left ventricle.

Take-Home Messages:

Relief of LVOT obstruction can be associated with paradoxical pulmonary congestion despite apparent left-sided unloading. This observation highlights the importance of comprehensive noninvasive hemodynamic assessment during the early treatment phases of obstructive HCM.

キーワード:

LVOT 拡張期機能不全肥大型心筋症マバカムテン肺うっ血

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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet

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Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension

Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension

Published on: February 11, 2017

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