What is the clinical evidence from this study?

Study design: Case Report. Population: Right ventricular endomyocardial fibrosis (n=4). Intervention: Surgical endocardial resection and tricuspid valve replacement. Primary outcome: Clinical course and survival.

What does this research mean for the field?

Echocardiography, angiocardiography, and computed tomography provide reliable diagnostic confirmation of right ventricular endomyocardial fibrosis, for which surgical endocardial resection and valve replacement can achieve long-term survival. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To describe the clinical manifestations, invasive and noninvasive diagnostic findings, and surgical management of patients with right ventricular endomyocardial fibrosis.

January 1, 1990Japanese Heart JournalOpen Access

Right ventricular endomyocardial fibrosis diagnosis and management.

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Key result

Surgical endocardial resection yields long-term clinical improvement in select patients with RV endomyocardial fibrosis.

Why the study?

Noninvasive and invasive diagnostic findings and management strategies for right ventricular endomyocardial fibrosis require detailed characterization to differentiate from other restrictive cardiac disorders.

Key Points

To describe the clinical manifestations, invasive and noninvasive diagnostic findings, and surgical management of patients with right ventricular endomyocardial fibrosis.
Evaluated 4 patients (aged 16–50 years) in NYHA functional class III–IV using clinical examination, electrocardiography, chest radiography, and invasive hemodynamic monitoring.
Performed cardiac imaging including echocardiography, angiocardiography, and computed tomography to assess cavity morphology and guide surgical resection.
All 4 patients exhibited elevated central venous pressure with prominent a- and v-waves, right atrial enlargement, and a right-sided diastolic plateau exceeding left ventricular end-diastolic pressure.
Multimodality imaging demonstrated obliteration of the right ventricular cavity concentrated at the apex across all cases.
Of the surgical candidates, 2 patients who underwent endocardial resection with tricuspid valve replacement survived long-term at 8 and 9 years postoperatively, while 1 died early post-surgery and 1 died before intervention.

Study Design

Type

Case Report (n=4)

Multicenter

Yes

Structured PICO

Population

4 male patients aged 16 to 50 years with severe right heart failure due to right ventricular endomyocardial fibrosis.

Intervention

Endocardial resection and tricuspid valve replacement (performed in 3 patients)

Outcome

Clinical course and survival

Noninvasive and invasive imaging modalities are diagnostic for right ventricular endomyocardial fibrosis, and surgical intervention can provide long-term survival.

Limitations

Very small sample size (4 patients)
Retrospective observational case series design
Endomyocardial biopsy was falsely negative in the two cases where it was performed

Discussion

No takes yet. Share an insight, caveat, or question.

Implication

Case series aids recognition of endomyocardial fibrosis; leaves open prevalence and management in non-endemic regions.

Cite This Study

Stierle et al. (1990) conducted a case report in Right ventricular endomyocardial fibrosis (n=4). Surgical endocardial resection and tricuspid valve replacement was evaluated on Clinical course and survival. Echocardiography, angiocardiography, and computed tomography provided diagnostic confirmation of right ventricular endomyocardial fibrosis, and surgical endocardial resection yielded long-term clinical improvement in two of three operated patients.

synapsesocial.com/papers/6aaca34c5eac244839754285 https://doi.org/10.1536/ihj.31.329

Topics

Echocardiography

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Right ventricular endomyocardial fibrosis diagnosis and management.

Key Points

Study Design

Structured PICO

Limitations

Discussion

Implication

Cite This Study

Topics

Right ventricular endomyocardial fibrosis diagnosis and management.

Key Points

Study Design

Structured PICO

Limitations

Discussion

Implication

Cite This Study

Also Consider

Topics

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