Subaortic and mid-ventricular obstructive hypertrophic cardiomyopathy with an apical Aneurysm: a case report

Tomás Francisco Cianciulli,Dora Faustina Serans,Alejandro Mario García Escudero,María Cristina Saccheri,Isabel Victoria Konopka,Rafael Salvador Acunzo,Horacio Alberto Prezioso,Osvaldo Horacio Masoli

doi:10.1186/1476-7120-4-15

Tomás Francisco Cianciulli, Dora Faustina Serans + Show 6 more

Open Access

https://doi.org/10.1186/1476-7120-4-15

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Journal: Cardiovascular ultrasound	Publication Date: Mar 1, 2006
Citations: 11	License type: CC BY 2.0

Affiliation: Argerich Hospital

Abstract

BackgroundMost patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction. Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm. An even more rare presentation is the coexistece mid-ventricular and apical HCM. This case is a combination of obstructive HCM with mid-ventricular HCM and an apical aneurysm, which to date, has not been reported in the literature.Case presentationThe patient is a 49 year-old lady who presents a combination of septal asymmetric hypertrophic cardiomyopathy (HCM) and midventricular HCM, a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, plus an apical aneurysm. Her clinical presentation was an acute myocardial infarction in June 2005. One month after hospital discharge, the electrocardiogram (ECG) showed a right bundle branch block (RBBB) with no Q waves or ST segment elevation. Coronary angiography revealed normal coronary arteries, left ventricular hypertrophy and an apical aneurysm.ConclusionThis case is a rare example of an asymptomatic patient with subaortic and mid-ventricular hypertrophic cardiomyopathy, who presents a myocardial infarction and normal coronary arteries, and during the course of her disease develops an apical aneurysm.

Highlights

Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction
Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm
An even more rare presentation is that of coexistent mid-ventricular and apical HCM. This case represents a combination of asymmetric septal and mid-ventricular HCM with an apical aneurysm, which to date, has not been reported in the literature

Summary

Background

HCM has a prevalence of 0.2 % (1 in 500) in the general population [1] and presents with marked clinical polymorphism, given by the extent and degree of hypertrophy of the myocardium involved and by the location and magnitude of the intraventricular gradient. The patient is a 49 year-old lady, with a family history of HCM and no coronary risk factors, who in June 2005 was admitted to the coronary care unit due to an episode of oppressive and prolonged chest pain Her ECG at admission showed sinus rhythm, left ventricular hypertrophy and ST segment elevation in leads V4 to V6, which later normalized, without developing Q waves (Figure 1A). The Doppler-echocardiogram showed a combination of asymmetric septal and mid-ventricular HCM (Figure 2), and the anterobasal septum measured 21 mm. The ECG at hospital discharge showed sinus rhythm, right bundle branch block (RBBB) with no Q waves, right ventricular hypertrophy and/or posterior necrosis (Figure 1B). Hemodynamic study revealed septal and mid-ventricular thickening (Figure 5) with normal epicardial coronary arteries, and apical aneurysm. During follow-up, the patient was treated with beta-blockers and remained clinically stable

Discussion

Conclusion

Findings

Maron BJ