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A 49 year-old woman with persisting LV obstruction involving the mitral valve apparatus, after surgical myectomy

A 49 year-old woman was admitted to our hospital for further evaluation and treatment stratification. At the time of hospitalization she suffered from changing dyspnea class III – IV with symptoms at rest. Clinical symptoms deteriorated typically after meals and after standing up. Furthermore, she reported on angina at mild exertion and sometimes at rest, pre-syncopal attacks independent from exercise, palpitations, and attacks of irregular heart beats. Medication at admission was 120 mg verapamil twice per day.
Myocardial Disease


HOCM was primarily diagnosed 18 months before admission to our hospital due to deteriorating symptoms of dyspnea. During that time, intraventricular pressure gradient was 65 mmHg at rest. Due to suspicion of an additional subvalvular membrane she was sent to surgery for myectomy and extirpation of the membrane. Surgery was performed without hemodynamic and clinical success.

Family history was negative regarding HCM and sudden cardiac death. Risk factor stratification showed flat increase of blood pressure during stress test with increase from 110/70 mmHg at rest to 120/60 mmHg at 70 Watts, pre-syncopal attacks, but no NSVT at holter-monitoring and maximal left ventricular wall thickness of 20 mm.

Physical examination showed a mildly overweight 49 year old woman (height 1.63 m, weight 70 kg, BMI 26.4 kg/m2) with a 3/6 typical systolic murmur at 5L2 with increase at Valsalva’s manoeuvre.

ECG at admission (Fig. 1) showed sinus rhythm without signs of LV hypertrophy and no LBBB after surgical myectomy.

Transthoracic echo (Fig. 2) showed typical HOCM with maximal septal thickness of 20 mm, SAM III° with resting gradient of 75 mmHg and provocable gradient of 125 mmHg. Obstruction was also detectable at the level of the papillary muscle. Mild to moderate mitral regurgitation was present - SAM associated and posteriorly directed. At TTE and TEE no subvalvular membrane could be identified.

Holter monitoring showed permanent sinus rhythm with 67 -117 beats / minute and no supraventricular or ventricular tachycardia. Cardiopulmonary exercise testing on a bicycle was performed and the patient reached 72 Watts after 8 minutes. Maximal oxygen uptake (peak VO2) was reduced to 16.6 ml/kg/min (66% predicted peak VO2).




Fig. 1: ECG without signs of LV hypertrophy and LBBB after myectomy.


Fig. 2. Baseline echo in diastole and systole ( parasternal long axis and 4CV ) after surgical myectomy with persisting LV obstruction due to SAM and involvement of the mitral valve apparatus.

What is your therapeutic strategy?

The resolution of the clinical case

A 49 year-old woman was admitted to our hospital for further evaluation and treatment stratification. At the time of hospitalization she suffered from changing dyspnea class III – IV with symptoms at rest. Clinical symptoms deteriorated typically after meals and after standing up. Furthermore, she reported on angina at mild exertion and sometimes at rest, pre-syncopal attacks independent from exercise, palpitations, and attacks of irregular heart beats. Medication at admission was 120 mg Verapamil twice per day.

HOCM was primarily diagnosed 18 months before admission to our hospital due to deteriorating symptoms of dyspnea. During that time, intraventricular pressure gradient was 65 mmHg at rest. Due to suspicion of an additional subvalvular membrane she was sent to surgery for myectomy and extirpation of the membrane. Surgery was performed without hemodynamic and clinical success.

Gradient reduction for symptomatic treatment

Therapeutic options for gradient reduction and symptomatic improvement are medical treatment (in Germany betablocker and verapamil), surgical myectomy and septal ablation (PTSMA). In the case, surgical myectomy and additional verapamil medication did not result in effective gradient reduction. Due to the severe clinical symptoms we discussed the alternative treatment options of gradient reduction. Finally, percutaneous septal ablation were performed with hemodynamic good result (Fig. 1 and 2). An AV-block III° was induced. Due to the facts that total heart block persisted and 2 weak risk factors of sudden cardiac death existed we implanted a two chamber ICD

Angiographic sequence of PTSMA

Fig. 1: Angiographic sequence of PTSMA with estimated and ablated septal branch (arrows). (PT = Pigtail catheter; PM = temporary pacemaker lead)


 

Hemodynamic result of PTSMA

Fig. 2: Hemodynamic result of PTSMA with complete elimination of the left ventricular gradient and induction of AV-block III° with necessary VVI stimulation of the temporary pacemaker.



















Clinical and echocardiographic follow-up

Clinical follow-up for 5 years after PTSMA is uneventful. The patient reported on an ongoing improvement of symptoms of functional class I-II which is mainly due to diastolic dysfunction. Angina pectoris and syncopal attacks disappeared. Echocardiographic follow-up studies (Fig. 3a and 3b) showed a typical septal thinning and ongoing gradient elimination at rest and provocation. Actual daily medication is 5 mg bisoprolol and 8 mg valsartan.

Fig. 3: Baseline echo 18 months after surgical myectomy (Fig. 3a) and 5 years follow-up echo 5 years after additional PTSMA (Fig 3b) in diastole and systole ( parasternal long axis and 4CV ). Baseline echo after surgical myectomy with persisting LV obstruction due to SAM and involvement of the mitral valve apparatus. Follow-up echo with septal thinning and complete elimination of the intracavitary gradient.








Summary

In this case we could demonstrate that myectomy and PTSMA are complementary treatment options for gradient reduction in HOCM. Surgical myectomy which was performed in addition to extirpation of a subvalvular membrane failed to reduce intracavitary obstruction. Although this obstruction was detectable at the level of the papillary muscle in addition to typical SAM association echo-guided PTSMA resulted in long-lasting gradient elimination. The observed complete heart block occurs in up to 10% of ablation-alone procedures with an increased probability up to 50% after prior myectomy.
In the last 15 years of experience total heart block with consecutive pacemaker implantation remains the most important complication as hospital mortality could be reduced to nearly 0% in experienced centers with knowledge of the special problems and complexities that can emerge in the postinterventional period. In our own series 750 consecutive patients had been treated without hospital death since February 2001.

Conclusion:

In conclusion, this case could clearly demonstrate that PTSMA and surgical myectomy are complementary treatment options in symptomatic HOCM patients, even in complex anatomic situations. Both, septal ablation as well as surgical myectomy should be limited to experienced centers in order to improve results and to reduce complications.
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.