Background: The natural history of Hypertrophic Obstructive Cardiomyopathy (HOCM), is well known from earlier investigations. The yearly death rate of medically or nontreated patients with HOCM is between 1.7% and 4%. After conservative management with beta-blockers and/or calcium antagonist, early improvement is followed in many patients by a symptomatic and clinical impairment, which today may lead to surgical or interventional treatment. Methods: From 1963 to 12/1998 a total of 519 patients were operated by transaortic subvalvular myectomy (TSM). The mean age was 49 +/- 11 years (range 3 months - 82 years) in 292 males and 227 females. Results: The early risk was related to the clinical class (NYHA) and the need for additional cardiac procedures during the same intervention. Total early mortality was 4.4% (n = 23), in isolated myectomy 3.6% (n = 11). During the last 10 years it could be reduced to 1.9%. The first complete (100%) reinvestigation of 346 patients up to 26 years after surgery (1963 - 1991) demonstrated a disease-related mortality rate of 5.2% (n = 20). The analysis of late deaths showed that disease-related lethal complications (sudden death, life-threatening arrhythmias, Valve endocarditis, secondary LV dilatation) were relatively rare, the age-related death rate nearly followed the natural course because of other causes. The cumulative survival rate after 10 years was 88%, after 20 - 26 years 72%. The yearly disease-related death rate could be reduced to 0.6%. The long-lasting, symptomatic clinical improvement (NYHA I-II), and also the physical and mental capacity with enlargement of the acitivity radius and improvement of quality of life were remarkable. The positive effects of surgical enlargement of the LVOT could be confirmed in the meantime by hemodynamic, rhythmological, echocardiographic investigations as well as endurance tests. Conclusion: We have examined the outcome of a large series of patients treated surgically for HOCM since 1963. The majority of patients were in NYHA class III and came to surgery after long-term medical, but finally insufficient, management. The perioperative risk could be reduced considerably during recent years, despite the advanced cardiomyopathy status. The long-term postoperative observation of the patients demonstrated an unexpectedly continuing good outcome. Therefore these results may serve as a standard for assessing the results after the less invasive alcohol-induced transcoronary ablation of septal hypertrophy.

Einleitung: Diese Studie hatte die simultane Erfassung von systolischer und diastolischer Funktion bei Patienten mit hypertroph-obstruktiver Kardiomyopathie und medikamentös therapierefraktären Beschwerden vor und nach Myektomie (M) zum Ziel. Methode: Hierzu wurden 19 Patienten (Alter 45 ± 16 Jahre, NYHA: 2,8 ± 0,3) 3,5 ± 3,6 Monate vor und 7,2 ± 4,5 Monate nach M mittels Echokardiographie, Swan-Ganz-Thermodilutionskatheter und simultaner Radionuklidventrikulographie untersucht. Resultate: Die Operation führte zu einer Reduktion von Ruhe- (40,1 ± 43,3 versus 7,6 ± 12,0 mm Hg, p ≤ 0,005) und Provokationsgradienten (92,4 ± 67,1 versus 21,3 ± 26,5 mm Hg, p ≤ 0,001). Die Beschwerdesymptomatik nach NYHA verbesserte sich auf 1,7 ± 1,4 (p ≤ 0,001). Die echokardiographisch bestimmte basale Septumdicke nahm ab (24,9 ± 6,3 versus 20,1 ± 6,8 mm; p ≤ 0,5), der enddiastolische Durchmesser stieg an (40,4 ± 5,2 versus 44,8 ± 7,1; p ≤ 0,05). Ebenso nahm die ergometrische Belastbarkeit von maximal erreichten 64,5 ± 19,2 auf 89,5 ± 24,0 Watt zu (p ≤ 0,001). Postoperativ fanden sich bei den systolischen Parametern eine verlängerte isovolumetrische Kontraktionszeit (65 ± 39 versus 112 ± 50 ms, p ≤ 0,01), eine verlängerte Zeit bis zur maximalen Ejektionsgeschwindigkeit (109 ± 40 versus 188 ± 42 ms, p ≤ 0,001) und in Ruhe eine verminderte linksventrikuläre globale Ejektionsfraktion (72 ± 12 versus 64 ± 11 % p ≤ 0,01). In Ruhe zeigte sich in den septalen und basalen Ventrikelanteilen und unter Belastung nur in den septalen Bereichen eine signifikante Minderung der Kontraktionen (p ≤ 0,05). Die Steigerungsfähigkeit der globalen und regionalen Ejektionsfraktionen unter Belastung war postoperativ nicht vermindert. Postoperativ zeigte sich unter Belastung ein signifikant größerer enddiastolischer Volumenindex (63 ± 19 versus 72 ± 17 ml * m−2, p ≤ 0,05). Der mittlere pulmonalkapilläre Verschlußdruck war sowohl in Ruhe (11,8 ± 3,8 versus 8,6 ± 2,4 mm Hg, p ≤ 0,05) als auch unter Belastung vermindert (27,0 ± 7,1 versus 20,4 ± 6,8 mm Hg, p ≤ 0,01). Schlußfolgerung: Patienten mit HOCM zeigen nach Myektomie eine deutliche Besserung der Belastungsfähigkeit. Die Operation geht mit einer Abnahme der basalen Septumdicke und einer Erweiterung des basalen enddiastolischen LV-Durchmessers einher. Konsekutiv-hämodynamisch ergibt sich eine modifizierte systolische Funktion mit einer septal-basal verminderten Ejektionsfraktion, eine Reduktion des intraventrikulären Gradienten begleitet von einer Besserung der diastolischen Funktion mit vermindertem Füllungsdruck bei vergrößertem enddiastolischem Volumen auf maximal erreichter Belastungsstufe.

INTRODUCTION:In this study systolic and diastolic function in patients with hypertrophic obstructive cardiomyopathy (HOCM) and intractable complaints to medication were investigated before and after a mean of 7 months after myectomy.METHODS:Investigations in 19 patients with HOCM included echocardiography, Swan-Ganz-thermodilution-catheter and radionuclide-angiography.RESULTS:Myectomy resulted in a reduction of the intraventricular gradient at rest (40.1 +/- 43.3 versus 7.6 +/- 12.0 mm Hg, p < or = 0.005) and under provocation (92.4 +/- 67.1 versus 21.3 +/- 26.5 mm Hg, p < or = 0.001). Echocardiographically determined basal septal thickness was reduced (24.9 +/- 6.3 versus 20.1 +/- 6.8 mm; p < or = 0.05) and diastolic diameter increased (40.4 +/- 5.2 versus 44.8 +/- 7.1; p < or = 0.05). Exercise tolerance increased from the maximally achieved 64.5 +/- 19.2 to 89.5 +/- 24.0 W (p < or = 0.001). Symptomatic status (NYHA) improved (1.7 +/- 1.4 versus 2.8 +/- 0.3 (p < or = 0.001). Systolic parameters showed at rest an increase in isovolumetric contraction time (65 +/- 39 versus 112 +/- 50 ms, p < or = 0.01), in time to peak ejection (109 +/- 40 versus 188 +/- 42 ms, p < or = 0.001), and a reduced left ventricular ejection fraction (72 +/- 12 versus 64 +/- 11%, p < or = 0.01). Analysis of regional ejection fraction revealed a significant reduction of ejection fraction in the basal septal region (p < or = 0.05). Increase of global and regional ejection fraction under exercise was still preserved. Mean pulmonary capillary wedge pressure was significantly reduced at rest (11.8 +/- 3.8 versus 8.6 +/- 2.4 mm Hg, p < or = 0.05) as well as under exercise (27.0 +/- 7.1 versus 20.4 +/- 6.8 mm Hg, p < or = 0.01), whereas left ventricular enddiastolic volume index (63 +/- 19 versus 72 +/- 17 ml*m-2, p < or = 0.05) was significantly increased.CONCLUSIONS:In patients with HOCM, myectomy reduces intraventricular gradient, increases exercise capacity, and is accompanied by improved diastolic function parameters, while systolic function parameters are generally reduced.

To determine the hemodynamic effects of postoperative left bundle branch block (LBBB) in patients with hypertrophic obstructive cardiomyopathy (HOCM), we investigated 28 patients using Swan-Ganz pulmonary artery catheterization at rest and on exercise. Fourteen patients had postoperative LBBB (group A) and 14 had undisturbed intraventricular conduction (group B). All patients were examined by clinical investigation, electrocardiogram and bicycle ergometer exercise preoperatively and postoperatively (mean 6 months). Pulmonary artery pressure and pulmonary capillary wedge pressure were continuously measured, stroke volume index and cardiac index were obtained by the thermodilution method. All patients showed an improvement of their clinical symptoms (NYHA class: 2.8 +/- 0.45 before and 1.7 +/- 0.22 after operation) (P < 0.05). The postoperative exercise capacity (Watt) increased significantly (P < 0.05) in group A by 38% and in group B by 30%. The maximum mean pulmonary artery pressure on physical exercise decreased in group A from 40.7 +/- 9.1 to 32.5 +/- 8.7 mmHg and in group B from 42.8 +/- 12.4 to 32.4 +/- 9.2 mmHg (P < 0.05). The maximal stroke volume index and cardiac index improved significantly in 9 of 14 patients in each group. Thus, patients with HOCM and LBBB after myectomy have a marked improvement in postoperative hemodynamics, the results equaling those of patients with undisturbed intraventricular conduction.

Pulsed Doppler echocardiography has been repeatedly used to assess left ventricular filling in cardiac diseases associated with left ventricular hypertrophy. Abnormalities in the transmitral flow velocity curve have been found in symmetrical left ventricular hypertrophy due to pressure overload, such as arterial hypertension and aortic stenosis [1], as well as in hypertrophic cardiomyopathy [2–4], a disease characterized by an asymmetrical ventricular hypertrophy of unknown etiology. Although hypertrophic cardiomyopathy is the disease with the most severe expressions of hypertrophy in human beings, Doppler studies of transmitral flow velocity failed to consistently demonstrate the signs of impaired left ventricular filling, especially in the obstructive form of the disease [3,4]. Several possible factors have been discussed to explain that [3–5], but no attention has been paid to the influence of the position of the sample volume. Based on observations in individual patients with hypertrophic cardiomyopathy, showing impressive variations of the diastolic flow velocity curve with different positions of the sample volume along the left ventricular inflow tract, we prospectively examined, whether the specific asymmetrical left ventricular geometry in hypertrophic cardiomyopathy could have an impact on regional differences in diastolic flow velocity patterns, when compared to symmetrical forms of hypertrophy.

Abnormal, dysplastic intramyocardial arteries were reported in autopsied hearts of hypertrophic cardiomyopathy. To elucidate their significance, the operatively-excised myectomy specimens of 24 patients with hypertrophic-obstructive cardiomyopathy (HOCM), of 18 patients with valvular aortic stenosis and of 10 postmortem normal hearts were investigated. Eight patients with HOCM had dysplastic intramyocardial arteries (greater than 100 microns external diameter) as well as dysplastic arterioles (less than 100 microns external diameter). The value of the scores for the thickness and fibroelastosis of the media was nearly doubled, the tunica intima was frequently thickened, and the lumen was relatively reduced in dysplastic vessels. Neither in controls nor in aortic stenosis dysplastic arteries were found. Volume density of patchy fibrosis (scars) was increased in patients with dysplastic arterial vessels (HOCM II) (7.2 +/- 4.4 Vv%) (p less than or equal to 0.05) as compared with HOCM without dysplastic vessels (HOCM I) (0.8 +/- 2.3 Vv%), with aortic stenosis (0.9 +/- 1.6 Vv%) or with controls (0 Vv%), Patients with HOCM II were significantly (p less than or equal to 0.05) younger (30 +/- 13 years) than those with HOCM I (53 +/- 12 years), aortic stenosis (56 +/- 12 years), or controls (63 +/- 21 years). The anterior septum was significantly thicker in HOCM II (29 +/- 7 mm) than in HOCM I (22 +/- 4 mm), in aortic stenosis (19 +/- 3 mm), or in controls (12 +/- 2 mm). Syncopes were complained by about 75% (6/8) of patients in HOCM II, by 54% (9/16) in HOCM I, and by 44% (8/18) in aortic stenosis (not significant).(ABSTRACT TRUNCATED AT 250 WORDS)

Objective. We investigated whether the site and severity of an obstruction in hypertrophic cardiomyopathy can be accurately predicted by the combined use of color-coded and continuous wave Doppler echocardiography.Background. Predicting the site of obstruction by end-systolic cavity shape is not reliable. Therefore, hemodynamic localization of the obstruction is required before surgery is performed. Such localization should be possible with color flow imaging, which provides two-dimensional velocity mapping reflecting the distribution of pressures within the left ventricle. Discrepancies in assessment of the pressure gradient by Doppler echocardiography and cardiac catheterization (which are usually not performed simultaneously) may be due to spontaneous variation of the dynamic obstruction in addition to technical factors related to both methods.Methods. Twenty consecutive patients with hypertrophic cardiomyopathy were examined 1 day before transseptal left heart catheterization. The obstruction site was defined by color flow mapping. The pressure gradient was determined by continuous wave Doppler echocardiography. Measurements were also performed simultaneously in 10 patients during cardiac catheterization.Results. Midventricular obstruction was correctly identified in 4 patients and subvalvular obstruction in 15 patients. One patient had no obstruction at rest. Invasively and noninvasively determined pressure gradients correlated well (r = 0.89, SEE = 16.3 mm Hg). Multiple single-beat analysis in 10 patients, also simultaneously examined with Doppler echocardiography and catheterization, yielded an excellent correlation (r = 0.97, SEE = 13.1 mm Hg). Comparing the simultaneous (r = 0.96, SEE = 12.5 mm Hg) and nonsimultaneous (r = 0.81, SEE = 23.8 mm Hg) recordings in these patients, we found that the spontaneous variation of the dynamic obstruction mainly accounted for discrepancies (p < 0.05).Conclusion. The combined use of color-coded and continuous wave Doppler echocardiography provides the relevant hemodynamic information required for decision-making in patients with hypertrophic cardiomyopathy who are considered for transaortic myectomy.

The influence of the extent and distribution of the echocardiographically determined left-ventricular hypertrophy was evaluated in respect to clinical symptoms and hemodynamics at rest and under bicycle-exercise. 49 patients with hypertrophic obstructive cardiomyopathy [HOCM] (34 men, 15 women, mean age 43 years, subvalvular gradient after provocation 10-270 mm Hg, ejection fraction > 55 %) and 10 controls (seven men, three women, mean age 44 years) were investigated. Enddiastolic left-ventricular diameter and basal thickness of the anterior and posterior septum, and the lateral and posterior wall of the left ventricle were echocardiographically measured. The distribution of basal hypertrophy (for the anterior septum and the posterior wall greater-than-or-equal-to 15 mm, for the posterior septum and the lateral wall greater-than-or-equal-to 17 mm) was classified as type I (1 segment, n = 17), II (2, n = 14) and III (greater-than-or-equal-to 3 segments, n = 18), according to Maron et al. (1981). At rest and during symptom-limited bicycle exercise, cardiac index (CI), stroke volume index (SVI), and mean pulmonary capillary wedge pressure (PCWP) were determined at sinus rhythm by a Swan-Ganz-catheter and by the thermodilution method.Results: With increasing hypertrophy, there was a reduction of the left-ventricular enddiastolic diameter. A NYHA-class > II was observed in 18% of patients with type-I hypertrophy, in 29 % with type II, but in 61 % with type III (p less-than-or-equal-to 0.05). Of patients with type I 6 % had suffered syncopes; with type II, 14 %, and with type III, 44% (p less-than-or-equal-to 0.05). In type-III hypertrophy the maximum exercise level (72 +/- 24 Watt) was significantly (p < 0.05) lower compared with controls (110 +/- 42 W), in type I (99 +/- 35 W), and in type II (100 +/- 45 W). At maximum stress PCWP was significantly increased in types I, II, and III (type I = 22 +/- 8 mm Hg, II = 24 +/- 12 mm Hg, III = 29 +/- 8 mm Hg) compared with controls (8 +/- 4 mm Hg); SVI was significantly lower in type III (49 +/- 13 ml/m2) than in controls (65 +/- 12 ml/m2) and in type I (60 +/- 11 ml/m2); SVI Was 60 +/- 17 ml/m2 in type II. At maximum stress, cardiac index was significantly lower in type III (6.2 +/- 2.1 l/min/m2) than in controls (8.9 +/- 2.1 l/min/m2) and in type 1 (8.4 +/- 1.7 l/min/m2).Conclusions: In patients with HOCM, severe left-ventricular hypertrophy type III accompanied by a reduced ventricular cavum is associated with more pronounced clinical symptoms, a reduced working capacity and the occurrence of syncopes. In these patients, despite increased filling pressures, the increase in stroke volume and cardiac index are insufficient at stress. Therefore, echocardiographically evaluated left-ventricular hypertrophy and geometry are helpful to estimate the severity of functional disorder in HOCM.

Surgical treatment of hypertrophic obstructive cardiomyopathy (HOCM), which may present in a typical (subaortic) or atypical (mid-ventricular) form, is indicated only after prior long-term drug treatment. The results obtained in 353 patients presenting with a symptomatic form of HOCM operated on between 1963 and 30 June 1991 are reported. The operative procedure took the form of transaortic subvalvular myectomy (TSM) as described by Morrow, modified by extending the myectomy. The patients comprised 210 males and 143 females aged between 6 and 76 years (average age 41.7 years). With few exceptions, all patients were in clinical stage III or IV (NYHA). The overall hospital mortality rate was 4.8% (n = 17); for TSM alone 3.1% (n = 8 out of 261 patients), for combination surgery with additional surgical measures 9.8% (n = 9 out of 92 patients). Among the last 194 patients (since 1984), the mortality rate was 2.06% (n = 4). To date, follow-up show an improvement in the symptoms and physical performance, and an annual mortality rate of about 1.8-4% among patients treated with drugs, and a post-operative mortality of about 1.1%, so that in HOCM patients with symptoms despite prior drug treatment, surgery can be recommended.

UNLABELLED:The influence of the extent and distribution of the echocardiographically determined left-ventricular hypertrophy was evaluated in respect to clinical symptoms and hemodynamics at rest and under bicycle-exercise. 49 patients with hypertrophic obstructive cardiomyopathy [HOCM] (34 men, 15 women, mean age 43 years, subvalvular gradient after provocation 10-270 mm Hg, ejection fraction greater than 55%) and 10 controls (seven men, three women, mean age 44 years) were investigated. Enddiastolic left-ventricular diameter and basal thickness of the anterior and posterior septum, and the lateral and posterior wall of the left ventricle were echocardiographically measured. The distribution of basal hypertrophy (for the anterior septum and the posterior wall greater than or equal to 15 mm, for the posterior septum and the lateral wall greater than or equal to 17 mm) was classified as type I (1 segment, n = 17), II (2, n = 14) and III (greater than or equal to 3 segments, n = 18), according to Maron et al. (1981). At rest and during symptom-limited bicycle exercise, cardiac index (CI), stroke volume index (SVI), and mean pulmonary capillary wedge pressure (PCWP) were determined at sinus rhythm by a Swan-Ganz-catheter and by the thermodilution method.RESULTS:With increasing hypertrophy, there was a reduction of the left-ventricular enddiastolic diameter. A NYHA-class greater than II was observed in 18% of patients with type-I hypertrophy, in 29% with type II, but in 61% with type III (p less than or equal to 0.05). Of patients with type III, 44% (p less than or equal to 0.05). In type-III hypertrophy the with type I 6% had suffered syncopes; with type II, 14%, and with type III, 44% (p less than or equal to 0.05). In type-III hypertrophy the maximum exercise level (72 +/- 24 Watt) was significantly (p less than 0.05) lower compared with controls (110 +/- 42 W), in type I (99 +/- 35 W), and in type II (100 +/- 45 W). At maximum stress PCWP was significantly increased in types I, II, and III (type I = 22 +/- 8 mm Hg, II = 24 +/- 12 mm Hg, III = 29 +/- 8 mm Hg) compared with controls (8 +/- 4 mm Hg); SVI was significantly lower in type III (49 +/- 13 ml/m2) than in controls (65 +/- 12 ml/m2) and in type I (60 +/- 11 ml/m2); SVI was 60 +/- 17 ml/m2 in type II. At maximum stress, cardiac index was significantly lower in type III (6.2 +/- 2.1 l/min/m2) than in controls (8.9 +/- 2.1 l/min/m2) and in type I (8.4 +/- 1.7 l/min/m2).CONCLUSIONS:In patients with HOCM, severe left-ventricular hypertrophy type III accompanied by a reduced ventricular cavum is associated with more pronounced clinical symptoms, a reduced working capacity and the occurrence of syncopes. In these patients, despite increased filling pressures, the increase in stroke volume and cardiac index are insufficient at stress. Therefore, echocardiographically evaluated left-ventricular hypertrophy and geometry are helpful to estimate the severity of functional disorder in HOCM.

To assess the behavior of the subvalvular pressure gradient under physical exercise, 13 patients with obstructive hypertrophic cardiomyopathy were examined during upright bicycle ergometry by means of Doppler echocardiography. Additionally, right-sided cardiac catheterization was performed within 7 days. In 10 patients adequate Doppler tracings could be obtained during exercise. The Doppler-derived systolic pressure gradient increased from 75 +/- 24 to 140 +/- 42 mm Hg (p less than 0.0005). This was associated with an increase in the duration of the systolic mitral-septal contact from 59 +/- 21 to 136 +/- 28 ms (p less than 0.0005). Correlation between the pressure gradient and the duration of mitral-septal contact at rest and during exercise was good (r = 0.86), whereas correlation between the resting and exercise pressure gradient (r = 0.34) did not reach statistical significance. The increase in stroke volume during exercise, from 90 +/- 18 to 95 +/- 24 ml, was significant (p less than 0.05) but minimal. Therefore, only a moderate increase in systolic flow, from 205 +/- 54 to 268 +/- 78 ml/s (p less than 0.0005), was observed. Outflow tract resistance, defined as the ratio of the pressure gradient to systolic flow, increased from 0.38 +/- 0.11 to 0.57 +/- 0.24 mm Hg.s/ml (p less than 0.01). Thus, in a selected group of patients with hypertrophic cardiomyopathy a substantial increase in the maximal pressure gradient during upright bicycle ergometry was demonstrated in most patients. Exercise Doppler echocardiography may be valuable to assess the hemodynamic significance of obstruction in individual patients in a physiologic setting and has a potential to monitor the effect of therapeutic interventions.

Der diagnostische Beitrag echokardiographischer Untersuchungsmethoden bei der hypertrophen Kardiomyopathie besteht nicht nur in einem Informationsgewinn, der sonst nur mittels invasiver Methoden zu erzielen ist. Die Echokardiographie gewährt vielmehr wie kein anderes Verfahren auf einfachem Wege Einblick in die beiden entscheidenden Komponenten des pathophysiologischen Prozesses: die veränderte Morphologie und, damit in Zusammenhang stehend, die veränderte Hämodynamik.

Hypertrophic obstructive cardiomyopathy (HOCM) — a disease of unknown etiology — is characterized by a hypertrophied left ventricle (LV) with a relatively small cavity, an asymmetric subaortic and/or midventricular or apical septal hypertrophy which may cause a considerable systolic obstruction of the left ventricular outflow-tract (LVOT) and diastolic LV dysfunction by delayed relaxation and filling impairment. In many cases a mitral regurgitation of different degrees and clinical importance can be found. Data of the natural history of the disease clearly indicate a slow continuing clinical deterioration. Sudden death — mainly in younger age groups and often after acute physical exertion — is a typical complication. Patients are additionally endangered by arrhythmias, systemic embolism, endocarditis, and congestive heart failure (CHF). The most important diagnostic procedures are listed in (Table 1). In our experience, clinical examination, carotid pulse tracings, echocardiography, Doppler echochardiography, transseptal left heart catheterization, and cineangiocardiography are the most important diagnostic tools. Management of clinically symptomatic HOCM (Table 2) was started medically using β-adrenergic blocking agents (propranolol [16], mainly to prevent provocation of the obstruction by beta-agonists [59]). Later Kaltenbach et al. [22] reported a good clinical response by using the calcium antagonist verapamil. Antiarrhythmic drugs were introduced by Wigle et al. [50] (disopyramide) or by McKenna et al. [32] (amiodarone).

This case report describes a patient with an uncommon type of mitral incompetence caused by a perivalvular communication between the left ventricle (LV) and the left atrium (LA) masked by a considerable fibrotic subvalvular aortic stenosis, endocarditis and congestive heart failure (CHF). A 64 year old farmer with a history of a systolic murmur since childhood complaining of increasing fatigue and dyspnoea, temperature over 39 degrees C, and signs of CHF was admitted and transferred to a cardiological unit. Invasive examination and continuing clinical deterioration caused urgent transfer for surgery under suspicion of a decompensated hypertrophic obstructive cardiomyopathy. Clinical investigation revealed a decompensated subvalvular aortic stenosis and a mild mitral insufficiency. At surgery the advanced fibrotic subvalvular stenosis was resected. After coming off bypass severe mitral insufficiency was detected by intraoperative analysis of the simultaneous intracavitary-pressure tracings. A midsystolic maximum of a high V-wave of the LA-pressure tracing was suggestive of an unusual reason of the mitral insufficiency. Reexploration indicated a perivalvular broad communication from the LA groove to the LV with an otherwise normal mitral valve. The communication was closed using buttressed mattress-sutures. This uncommon type of mitral incompetence via a perivalvular LA-LV communication was probably caused by endocarditis and an intramyocardial abscess in the LA-wall which subendocardially led to LV-LA communication.

Cleland, in 1958 performed the first subvalvular myotomy for relief of hyper-trophie obstructive cardiomyopathy (HOCM); following him came a variety of surgical procedures. In our clinic we principally use the Morrow technique (subvalvular myectomy) which was modified for better initial pressure gradient reduction, improvement of diastolic relaxation, and decrease of concomitant mitral insufficiency. Since 1963 a total of 253 patients were operated upon for symptomatic HOCM (NYHA class III or IV) after failing medical therapy (β-blockers, calcium antagonists). Their mean age was 41–45 years (range, 6–74 years). The male/female radio was 1.7 : 1. In 230 patients a typical subvalvular significant obstruction was present, while 23 patients had an additional, atypical midventricular obstruction. The surgical steps for both variations of HOCM are described in detail. The hospital mortality for transaortic subvalvular myectomy was 3.2% (6 of 190 patients), while for patients needing additional cardiac procedures it was 12.7% (8 of 63 patients). Taking account of the natural history of HOCM involving continuing deterioration, all patients were followed postoperatively (100%). The yearly death rate (HOCM-related) was 1.2%. However, the majority of the surviving patients (more than 6 months) demonstrated considerable long-term clinical improvement regarding complaints, physical capacity, and hemodynamics. Thus, operative relief of symptomatic HOCM is of long-term benefit for the patients.

Progressive external ophthalmoplegia (PEO) is accompanied by a progressive weakness of the external muscles of the eyes. It was first described by Albrecht von Graefe in 1866 [20]. First believed to be of neurogenic origin, it was not until 1951 that Kiloh and Nevin [30] suggested that the ocular signs were due to a myopathy as they in one case found histological abnormalities in the medial rectus muscle.