Clinical course and prognosis of patients with typical and atypical hypertrophic obstructive and with hypertrophic non-obstructive cardiomyopathy

Abstract

In a total of 339 patients with hypertrophic cardiomyopathies (typical HOCM, n = 224; atypical HOCM, n = 30; HNCM, n = 80) the clinical course, the Sokolow-Lyon index in the ECG and the prognosis as demonstrated by cumulative survival rates were analysed to get more differentiated information for the characterization of these myocardial disorders. No change in the type of hypertrophic cardiomyopathy was found, indicating different clinical entities. No increase of the Sokolow-Lyon index was observed during follow-up. Only a small proportion of patients seemed to benefit from treatment with propranolol. Surgical treatment appears to be the therapy of choice, at least in the advanced stage of the disease refractory to medical treatment. In addition, strong evidence was obtained that surgical treatment improved the prognosis in patients with typical HOCM.

Role of Transcoronary Ablation of Septal Hypertrophy in Patients With Hypertrophic Cardiomyopathy, New York Heart Association Functional Class III or IV, and Outflow Obstruction Only Under Provocable Conditions

“…[1][2][3][4] The therapy most widely applied is surgical myectomy by removing a small amount of muscle from the basal interventricular septum. [5][6][7][8][9] Surgery substantially reduces the subaortic outflow gradient in Ͼ90% of patients and results in persistent symptomatic improvement in Ϸ70% to 90%.…”

“…[5][6][7][8][9] Surgery substantially reduces the subaortic outflow gradient in Ͼ90% of patients and results in persistent symptomatic improvement in Ϸ70% to 90%. [1][2]4,[7][8][9][10] In recent years, transcoronary ablation of septal hypertrophy (TASH) by selective transcatheter septal branch injection of ethanol has been shown to substantially reduce outflow obstruction in 80% to 90% and symptoms in 84% to 90% of patients. [11][12][13][14][15][16][17] Therefore, TASH may be an effective alternative to surgery.…”

Caso 01/2008: paciente jovem do sexo feminino, portadora de forma familiar de cardiomiopatia hipertrófica que evoluiu com síncopes, arritmia ventricular complexa e choque cardiogênico

“…The annual mortality in the reference centers, which usually include the symptomatic patients, is approximately 2% in adults, with values between 4 and 6% during childhood and adolescence [16][17][18][19][20] . The most prevalent causes of mortality are sudden cardiac death, HF and CVA 15 .…”

Hypertrophische Kardiomyopathie

“…Symptomatische Patienten mit HOCM beklagen Dyspnoe (60%), atypische Angina pectoris (58%), Palpitationen (39%), Schwindel (18%) und Synkopen (14%). Sofern eine mindestens 3-monatige medikamentöse Therapie (hochdosiert mit -Blockern oder Verapamil, kombinierte Gabe nicht empfehlenswert [10][11][12]) unzureichend effektiv (persistierend NYHA-Stadium III und IV, subjektiv deutliche Beeinträchtigung im NYHA-Stadium II, schwere Angina pectoris, rezidivierende Synkopen, Vorhofflimmern mit tachykarder AV-Überleitung, Ruhegradient > 75-100 mmHg) oder zur Vermeidung eines plötzlichen Herztodes unzureichend sicher ist [10][11][12], stehen interventionelle Verfahren zur Verfügung: 1. operative Myektomie [2,[9][10][11][12]19], 2. Schrittmacherbehandlung mit vorhofgesteuerter Kammerstimulation [2,[11][12][13]17], 3. transkoronare Ablation der Septumhypertrophie (TASH) [2, 5, 10-12], 4.…”

“…Für die operative Myektomie ist eine Abnahme von Ausflusstraktobstruktion, funktioneller Mitralinsuffizienz, Belastungsdyspnoe, Angina pectoris und Synkopenhäu-figkeit belegt [9][10][11][12]19]. Eine Steigerung der Belastbarkeit, der maximalen Sauerstoffaufnahme sowie eine Verbesserung des pulmonalarteriellen Drucks und des Cardiac Index (CI) unter Belastung objektivieren die Wirksamkeit [12].…”