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Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis.
BMC Cardiovasc Disord 2013; 13:43BC

Abstract

BACKGROUND

Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare.

CASE PRESENTATION

A 7-year-old boy was referred to a tertiary care hospital with complaints of dyspnea on moderate exertion and palpitations of about 2 years duration. Physical examination revealed parasternal lift, systolic thrill and a 4/6 ejection systolic murmur, best heard over the left 2nd intercostal space. His oxygen saturation was 88% on room air. Two-dimensional echocardiography showed a thickened pulmonary valve with fused leaflets that show severe systolic doming. There was a discrete subpulmonic membrane about 1.3 cm below the pulmonary valve annulus. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 185 mmHg across the pulmonary valve. Balloon dilation of the pulmonary valve was performed and the pressure gradient came down to 50 mmHg. Follow-up transthoracic echocardiography showed residual pressure gradient of about 50-60 mmHg across the pulmonary valve. The residual pressure gradient appeared to be mainly subvalvar, as seen on the continuous wave Doppler tracing. The patient reported marked improvement in terms of exercise tolerance and subjective symptoms.

CONCLUSIONS

Association of subpulmonic membrane with severe pulmonary valvar stenosis, concordant ventriculoarterial connection and intact ventricular septum is rare. When it occurs, the result of percutaneous valve dilation may be suboptimal.

Authors+Show Affiliations

Department of Pediatrics & Child Health, School of Medicine, Addis Ababa, University & Cardiac Center, Addis Ababa, Ethiopia. endalet2008@gmail.comNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

23799957

Citation

Tefera, Endale, et al. "Discrete Subpulmonic Membrane in Association With Isolated Severe Pulmonary Valvar Stenosis." BMC Cardiovascular Disorders, vol. 13, 2013, p. 43.
Tefera E, Bermudez-Cañete R, Rubio L. Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis. BMC Cardiovasc Disord. 2013;13:43.
Tefera, E., Bermudez-Cañete, R., & Rubio, L. (2013). Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis. BMC Cardiovascular Disorders, 13, p. 43. doi:10.1186/1471-2261-13-43.
Tefera E, Bermudez-Cañete R, Rubio L. Discrete Subpulmonic Membrane in Association With Isolated Severe Pulmonary Valvar Stenosis. BMC Cardiovasc Disord. 2013 Jun 21;13:43. PubMed PMID: 23799957.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis. AU - Tefera,Endale, AU - Bermudez-Cañete,Ramón, AU - Rubio,Lola, Y1 - 2013/06/21/ PY - 2013/02/11/received PY - 2013/06/19/accepted PY - 2013/6/27/entrez PY - 2013/6/27/pubmed PY - 2014/2/4/medline SP - 43 EP - 43 JF - BMC cardiovascular disorders JO - BMC Cardiovasc Disord VL - 13 N2 - BACKGROUND: Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. CASE PRESENTATION: A 7-year-old boy was referred to a tertiary care hospital with complaints of dyspnea on moderate exertion and palpitations of about 2 years duration. Physical examination revealed parasternal lift, systolic thrill and a 4/6 ejection systolic murmur, best heard over the left 2nd intercostal space. His oxygen saturation was 88% on room air. Two-dimensional echocardiography showed a thickened pulmonary valve with fused leaflets that show severe systolic doming. There was a discrete subpulmonic membrane about 1.3 cm below the pulmonary valve annulus. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 185 mmHg across the pulmonary valve. Balloon dilation of the pulmonary valve was performed and the pressure gradient came down to 50 mmHg. Follow-up transthoracic echocardiography showed residual pressure gradient of about 50-60 mmHg across the pulmonary valve. The residual pressure gradient appeared to be mainly subvalvar, as seen on the continuous wave Doppler tracing. The patient reported marked improvement in terms of exercise tolerance and subjective symptoms. CONCLUSIONS: Association of subpulmonic membrane with severe pulmonary valvar stenosis, concordant ventriculoarterial connection and intact ventricular septum is rare. When it occurs, the result of percutaneous valve dilation may be suboptimal. SN - 1471-2261 UR - https://www.unboundmedicine.com/medline/citation/23799957/Discrete_subpulmonic_membrane_in_association_with_isolated_severe_pulmonary_valvar_stenosis_ L2 - https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/1471-2261-13-43 DB - PRIME DP - Unbound Medicine ER -