What is the clinical evidence from this study?

Study design: Case Report. Population: Fetal Primary Spongiform Cardiomyopathy (n=3). Intervention: Fetal primary spongiform cardiomyopathy.

What question did this study set out to answer?

To establish reliable prenatal diagnostic criteria for distinguishing rare fetal primary spongiform (noncompacted) cardiomyopathy from other forms of intrauterine heart disease.

May 1, 2002Obstetrics and Gynecology

Criterion for Fetal Primary Spongiform Cardiomyopathy

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Key result

Fetal primary spongiform cardiomyopathy is characterized early by a restrictive pattern with no ventricular enlargement and prominent atria, presenting with hydrops and bradycardia.

Why the study?

What are the early diagnostic characteristics of fetal primary spongiform cardiomyopathy?

Key Points

To establish reliable prenatal diagnostic criteria for distinguishing rare fetal primary spongiform (noncompacted) cardiomyopathy from other forms of intrauterine heart disease.
Clinical, echocardiographic, and postmortem autopsy evaluation of N=3 perinatal cases diagnosed with primary spongiform cardiomyopathy.
Assessment of cardiac chamber dimensions, ventricular trabeculation, contractile function, and associated fetal anomalies.
All three fetuses exhibited thickened and markedly trabeculated apical myocardium, enlarged atria, hydrops, and bradycardia despite having normal or only mildly diminished contractility.
Left ventricles were not dilated in two of three cases, and all three hydrops cases resulted in death, while the presentation of two females ruled out sex-linked Barth syndrome.

Study Design

Type

Case Report (n=3)

Structured PICO

What are the early diagnostic characteristics of fetal primary spongiform cardiomyopathy?

Population

Three perinatal cases of spongiform (noncompacted) cardiomyopathy evaluated with echocardiography and autopsy.

Outcome

Echocardiographic and autopsy findings to establish diagnostic criteriasurrogate

Fetal primary spongiform cardiomyopathy presents early as a restrictive cardiomyopathy with prominent atria and non-dilated ventricles, distinguishing it from dilated cardiomyopathy.

Discussion

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Implication

May aid early recognition of restrictive fetal cardiomyopathy in hydrops; case report leaves diagnostic criteria open for validation.

Cite This Study

Guntheroth et al. (2002) conducted a case report in Fetal Primary Spongiform Cardiomyopathy (n=3). Fetal primary spongiform cardiomyopathy was evaluated. Fetal primary spongiform cardiomyopathy is characterized early by a restrictive pattern with no ventricular enlargement and prominent atria, presenting with hydrops and bradycardia.

synapsesocial.com/papers/6a903a1de73a4c404fc9299e https://doi.org/10.1097/00006250-200205001-00004

View Full Paper

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Criterion for Fetal Primary Spongiform Cardiomyopathy

Key Points

Study Design

Structured PICO

Discussion

Implication

Cite This Study

Criterion for Fetal Primary Spongiform Cardiomyopathy

Key Points

Study Design

Structured PICO

Discussion

Implication

Cite This Study

Also Consider

Also Consider