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Review
. 2018 Dec;15(4):246-253.
doi: 10.5114/kitp.2018.80922. Epub 2018 Dec 31.

Apical hypertrophic cardiomyopathy: diagnosis, medical and surgical treatment

Jerzy Paluszkiewicz  1 , Beata Krasinska  2 , Hendrik Milting  3 , Jan Gummert  4 , Małgorzata Pyda  1
Affiliations
Review

Apical hypertrophic cardiomyopathy: diagnosis, medical and surgical treatment

Jerzy Paluszkiewicz et al. Kardiochir Torakochirurgia Pol. 2018 Dec.

Abstract
in English, Polish

Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy, occasionally resulting in severe complications. The paper covers the etiology and pathogenesis of AHCM, different imaging methods and characteristic appearance of the disease in each of them. Echocardiography and cardiovascular magnetic resonance imaging (CMR) are known to be the most valuable imaging methods. Moreover, this review presents medical and surgical treatment, as well as the clinical course and prognosis. Despite possible morbid events the overall cardiovascular mortality rate of AHCM patients is low, and the prognosis is relatively optimistic.

Kardiomiopatia koniuszkowa (AHCM) jest rzadką postacią kardiomiopatii przerostowej, która w niektórych przypadkach powoduje poważne komplikacje. Autorzy przedstawiają etiologię i patogenezę AHCM oraz różne techniki obrazowania pozwalające na ustalenie rozpoznania. Echokardiografia i magnetyczny rezonans jądrowy są najlepszymi metodami diagnostycznymi. Ponadto autorzy omawiają aktualne metody leczenia farmakologicznego i chirurgicznego, a także przebieg kliniczny i rokowanie. Pomimo występowania poważnych powikłań śmiertelność pacjentów z AHCM jest stosunkowo mała, a przebieg łagodny.

Keywords: apical hypertrophic cardiomyopathy; echocardiography; magnetic resonance imaging; sudden cardiac death.

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Figures

Fig. 1
Fig. 1
A – Transthoracic echocardiography, four-chamber view. Patient with AHCM. Note the increased thickness of the apex of the left ventricle (arrow). B – Transthoracic echocardiography, fourchamber view. Contrast echocardiography (SonoVue). The same patient as in Figure 1 A. Note the opacification of the left ventricle revealing typical silhouette of “ace of spades”. C – Transthoracic echocardiography, four-chamber view. Patient with AHCM. Contrast echocardiography (SonoVue). Note the opacification of the left ventricle revealing the presence of apical aneurysm (arrow) LA – left atrium, LV – left ventricle, RA – right atrium, RV – right ventricle.
Fig. 2
Fig. 2
A – Cardiac magnetic resonance, four-chamber view. Patient with AHCM. Note isolated apical hypertrophy (arrow) with apical aneurysm (dashed arrow). B – Cardiac magnetic resonance, four-chamber view. Patient with AHCM. Note apical fibrosis as revealed with gadolinium late enhancement (arrow) LA – left atrium, LV – left ventricle, RA – right atrium, RV – right ventricle.
Fig. 3
Fig. 3
Left ventricular angiogram in patient with known AHCM. Note the typical shape of the left ventricle with “ace of spades” silhouette Ao – ascending aorta, LV – left ventricle.
Fig. 4
Fig. 4
A – Transoesophageal echocardiography, X-plane view. Patient with AHCM. Note the increased thickness of the apex and small end-diastolic volume of the left ventricle. B – Transoesophageal echocardiography, three-chamber view. The same patient as in Figure 4 A. Note the increased thickness of the apex and small end-diastolic volume of the left ventricle Ao – ascending aorta, LA – left atrium, LV – left ventricle.
Fig. 5
Fig. 5
A – Intraoperative picture of opened apex of the left ventricle. Note thickened apex of the left ventricle (arrow). B – Intraoperative picture after apical myectomy. Note presence of Teflon patch (arrow) closing apex of the left ventricle
Fig. 6
Fig. 6
A – Transoesophageal echocardiography, three-chamber view. The same patient as in Figures 4 A, B after myectomy. Note the increased end-diastolic volume of the left ventricle. B – Transoesophageal echocardiography, two-chamber view. The same patient as in Figures 4 A, B after myectomy. Note the increased end-diastolic volume of the left ventricle Ao – ascending aorta, LA – left atrium, LV – left ventricle.
Fig. 7
Fig. 7
A – Transthoracic echocardiography, four-chamber view. The same patient as in Figures 4 A, B 12 months after myectomy. Note the increased end-diastolic volume of the left ventricle. B – Transthoracic echocardiography, two-chamber view. The same patient as in Figures 4 A, B 12 months after myectomy. Note the increased end-diastolic volume of the left ventricle LA – left atrium, LV – left ventricle, RA – right atrium, RV – right ventricle.
See this image and copyright information in PMC

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