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Image of the Month: Advanced Stage of Hypertrophic Obstructive Cardiomyopathy

Image presented by the Working Group on Myocardial and Pericardial Diseases

Presented by: Angelos G. Rigopoulos,1,2 Hubert Seggewiss,1 Ioannis C. Rizos2

11st Department of Internal Medicine, Leopoldina Hospital, Schweinfurt, Germany

22nd Department of Cardiology, University of Athens Medical School, Athens, Greece

Alcohol septal reduction in hypertrophic obstructive cardiomyopathy (HOCM) is indicated for patients with drug refractory symptoms or drug intolerance who have significant left ventricular obstruction.(1) On the other hand, several structural and functional changes including low-normal left ventricular (LV) ejection fraction, moderate to severe diastolic dysfunction, marked atrial dilatation, thinning of the LV walls, onset of atrial fibrillation, spontaneous reduction or loss of LV outflow obstruction, and LV apical aneurysms characterise an advanced stage of progression in the physical history of the disease which signifies adverse remodelling and clinical deterioration.(2) The benefit derived from septal reduction treatments in this stage is largely unknown.

A 35-year-old female obese patient (173cm, 91 Kg, BMI 30,4 Kg/m2) with diagnosis of familial hypertrophic cardiomyopathy since the age of 13 years was referred to the cardiomyopathy clinic due to increasing symptoms in the last 5 years: dyspnea NYHA III with retrosternal discomfort, orthopnea in the last weeks (needing 3 pillows at night), dizziness by standing up and frequent palpitations. Due to low blood pressure (100/60 mmHg) and sinus bradycardia (54/min) she could not tolerate negative inotropes (beta-blockers or verapamil) at an effective dosage.

Cardiopulmonary exercise test showed a reduced exercise capacity (89 W, 58% of the predicted value), reduced oxygen consumption (15.2 ml/Kg/min (69% of the predicted value) and a moderate increase in systolic blood pressure (from 90/60 to 120/70 mmHg).

Echocardiography showed nondilated left ventricle (LV enddiastolic diameter 42 mm, LVESD 22 mm) with preserved systolic function, restrictive transmitral flow pattern (Image 2) with remarkably dilated left atrium with low contractility (62 mm, area and volume at end-systole 51cm2, 262 cm3 and at end-diastole 46 cm2, 227 cm3 respectively). A significant LV outflow tract gradient (LVOTG) due to systolic anterior motion (SAM) of the mitral valve was measured at rest (42 mmHg) with increase during Valsalva (70 mmHg) and after a fortuitus ventricular extrasystolic beat (119 mmHg).

Heart catheterization measurements also showed a significant LVOTG at rest (50 mmHg), during Valsalva (90 mmHg) and postextrasystole (110 mmHg).

Left ventriculography provided a clear demonstration of left ventricular remodeling with hypokinesis of the anterolateral and apical segments that was difficult to appreciate with echocardiography.

Due to the increasing symptoms and the significant left ventricular outflow tract obstruction, we discussed the necessity of a septal reduction therapy with the patient and concluded to alcohol septal ablation. The intervention was performed with a standardized technique. (3) Myocardial contrast echocardiography proved that the second septal branch ideally perfused the basal part of the septum extending to the point of mitral-septal contact and 2 ml of alcohol was slowly injected in this branch after excluding any collateralization with other septal perforators (Image 1).

Image 1. Presentation of the alcohol septal ablation technique in the first (upper row) and the second (lower row) septal branch of the left anterior descending artery. The first image in each row shows the coronary arteriography of the left coronary artery during alcohol septal ablation while the middle and the right image show the apical 4-chamber view and the long axis parasternal view during myocardial contrast echocardiography (respectively). In the upper image row the first septal branch is chosen as target (1). After insertion of an over-the-wire balloon catheter and inflation of the balloon, echocardiographic contrast medium (Levovist®, Schering, Bayer, Germany) is injected through the central lumen of the balloon under simultaneous transthoracic echocardiography. In the apical 4-chamber view and the long axis parasternal view a small part of the basal septum is opacified, which was deemed not enough for a successful ablation. Thus, the second septal branch (2) was chosen as target branch (lower image row). Contrast echocardiography showed a larger opacified area covering most of the basal septum (albeit more to the right part). After alcohol injection, echocardiography confirmed the good choice by depicting a satisfactory alcohol depot (not shown).

The symptoms of the patient improved dramatically even in the first week (ΝΥΗΑΙΙwithout anginal symptoms, nocturnal dyspnea, or dizziness at standing up, and less frequent palpitation).

Image 2. Comparison between echocardiographic examination before(upper panel)and 3 months after alcohol septal ablation (lower panel). Doppler imaging of the transmitral flow, and measurement of the left atrial area and volume in end-systole and end-diastole.The reduction of the left atrial volumes with increase in left atrial systolic output (right column) reflects the obvious improvement in the diastolic function after alcohol septal ablation (left column).

Echocardiography at 3-month follow-up confirmed the good result with decrease of the gradients (20 mmHg at rest, 40 mmHg with Valsalva) and improvement of the mitral inflow pattern (Image 2). Left atrial enlargement markedly decreased (61 mm, area and volume at end-systole 43 cm2, 202 ml, and at end-diastole 33cm2, 134 m respectively) with now obviously improved atrial contractility.

The patient became pregnant 5 months after septal ablation and had no specific problems until the last trimester, when she appeared with paroxysmal atrial fibrillation (PAF) and wide QRS tachycardia. She stayed in hospital for rhythm monitoring and received an ICD soon after delivery of a healthy boy with caesarian section.

At 3-year follow-up, the patient has put on excess weight (110 kg) and appears to be in NYHA II with occasional palpitation without any documented PAF episodes. Echocardiography shows a stable result with no LVOTG (6mmHg, 17mmHg) and a preserved transmitral inflow pattern (DT 230 msec) as well as a more or less preserved left atrial geometry and function (volume at end-systole 213ml and at end-diastole 148 ml).

We conclude that despite an advanced stage of hypertrophic cardiomyopathy with excessive remodeling of the left ventricle and the left atrium, the existence of a significant obstruction identifies patients that can still benefit from septal reduction treatment by improving diastolic function(4) and probably delaying the rate of the remodeling process apart from symptomatic relief.

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.