Long-term follow-up of medical versus surgical therapy for hypertrophic cardiomyopathy: A retrospective study

In a retrospective analysis 139 patients with hypertrophic cardiomyopathy were followed up for 8.9 years (range 1 to 28 years). Patients were divided into two groups: Group 1 consisted of 60 patients with medical therapy and Group 2 of 79 patients with surgical therapy (septal myectomy). Groups 1 and 2 were subdivided according to the medical treatment. Group la received propranolol, 160 mg/day (n = 20); Group lb verapamil, 360 mg/day (n = 18); and Group 1c, no therapy (n = 22). Group 2a received verapamil, 120 to 360 mg/day, after septal myectomy (n = 17) and Group 2b had no medical therapy after surgery (n = 62).In Group 1, 19 patients died (annual mortality rate 3.6%) and in Group 2,17 patients died (mortality rate 2.4%, p = NS). Of the patients who died, approximately one half to two thirds in both Groups 1 and 2 died suddenly and the other one half to one third died because of congestive heart failure. The 10 year cumulative survival rate was 67% in Group 1, significantly smaller than that in Group 2 (84%, p < 0.05). In the subgroups, the 10 year survival rate was 67% in Group la, 80% in lb (p < 0.05 versus la) and 65% in lc (p < 0.05 versus 1b). The 10 year survival rate was 100% in Group 2a (p < 0.05 versus la, lb, 1c) and 78% in Group 2b (p < 0.05 versus 2a).It is concluded that cumulative survival rate is significantly better in surgically than in medically treated patients. However, the survival rate among medically treated patients was better in those treated with verapamil than in those treated with propranolol or in untreated patients. The 10 year survival rate was similar in the medically treated patients receiving verapamil (80%) and the entire surgically treated group (84%, p = NS). The most favorable outcome was observed in surgically treated patients receiving long-term therapy with verapamil, probably as a result of the reduction of systolic pressure overload by septal myectomy and improvement in left ventricular diastolic function mediated by verapamil

Emergency hospital admissions and three-year survival of adults with and without cardiovascular surgery for congenital cardiac disease

AbstractObjective:This study determined the quantity and nature of emergencies leading to unscheduled hospital admissions of adults with congenital cardiac disease and their mid-term survival.ResultsDuring 1 year, 429 adults with congenital cardiac diseases were admitted 571 times, and 124 admissions (22%) of 95 patients (22%) were emergency admissions. Fifteen of the 95 patients were seen for the first time in 1 of the participating centers. The underlying anomalies were Fallot's tetralogy and pulmonary atresia (n = 26/7), univentricular heart after Fontan procedure (n = 25), atrial septal defect (n = 18), Eisenmenger syndrome (n = 12), complete transposition (n = 11), and others (n = 25). Indications for admission were cardiovascular complications (n = 103; 83%) (arrhythmia, cardiac failure, syncope, pacemaker problems, pericardial tamponade, and sudden death), infections (n = 8, 6%) (endocarditis, pacemaker infection, pneumonia, and cerebral abscess), acute chest pain (n = 7; 6%), and acute abdominal pain (n = 4; 3%). All patients required immediate emergency care, and 16 patients (17%) required urgent cardiovascular or abdominal surgery. Six patients died during the hospital stay. During a follow-up of 2.9 years (SD 0.8), 16 (18%) of the discharged patients died, and 2 additional patients underwent heart or heart-lung transplantation.ConclusionAdults with congenital cardiac disease often experience serious emergency situations with a high in-hospital and mid-term post-hospital mortality. Care given by physicians with special expertise is important in this specific group of patients

Persistent diastolic dysfunction late after valve replacement in severe aortic regurgitation

BACKGROUND: Regression of left ventricular (LV) hypertrophy with normalization of diastolic function has been reported in patients with aortic stenosis late after aortic valve replacement (AVR). The purpose of the present study was to evaluate the effect of AVR on LV function and structure in chronic aortic regurgitation early and late after AVR. METHODS AND RESULTS: Twenty-six patients were included in the present analysis. Eleven patients with severe aortic regurgitation were studied before, early (21 months) and late (89 months) after AVR through the use of LV biplane angiograms, high-fidelity pressure measurements, and LV endomyocardial biopsies. Fifteen healthy subjects were used as controls. LV systolic function was determined from biplane ejection fraction and midwall fractional shortening. LV diastolic function was calculated from the time constant of LV relaxation, peak filling rates, and myocardial stiffness constant. LV structure was assessed from muscle fiber diameter, interstitial fibrosis, and fibrous content. LV muscle mass decreased significantly by 38% early and 55% late after surgery. Ejection fraction was significantly reduced preoperatively and did not change after AVR (P=NS). LV relaxation was significantly prolonged before surgery (89+/-28 ms) but was normalized late after AVR (42+/-14 ms). Early and late peak filling rates were increased preoperatively but normalized postoperatively. Diastolic stiffness constant was increased before surgery (22+/-6 versus 9+/-3 in control subjects; P=0.0003) and remained elevated early and late after AVR (23+/-4; P=0.002). Muscle fiber diameter decreased significantly after AVR but remained increased at late follow-up. Interstitial fibrosis was increased preoperatively and increased even further early but decreased late after AVR. Fibrosis was positively linearly correlated to myocardial stiffness and inversely correlated to LV ejection fraction. CONCLUSIONS: Patients with aortic regurgitation show normalization of macroscopic LV hypertrophy late after AVR, although fiber hypertrophy persists. These changes in LV myocardial structure late after AVR are accompanied by a change in passive elastic properties with persistent diastolic dysfunction

Long-term follow-up after thoracic radiotherapy: symptomatic heart disease is an ominous digne

Background: Thoracic radiotherapy (RT), especially with past technology, may affect the heart, but rarely leads to symptoms. In patients with symptomatic heart disease after RT, outcome seems to be dismal. Methods: In this observational descriptive study, clinical characteristics, findings of ECG, echocardiography, cardiac interventions and follow-up were analysed in patients with prior RT and symptomatic heart disease. The patients were identified in the echocardiography database during a ten year period. Results: There were 25 patients who had thoracic RT at a median age of 35 years (range: 9–59) for lymphoma (12 patients), breast cancer (9) or other cancer (4). At least likely inclusion of the heart in the target volume of previous RT was present in of 16 of 20 patients with detailed information on RT. Last follow-up was 24 years (range: 5–57) after RT at a median age of 56 years (range: 30–84). Symptoms (≥1 per patient) included: dyspnea (21 patients), angina (12) and/or heart failure (10). Three patients had prior myocardial infarction. The following disease was found: moderate valvular disease in 19 patients (76%), coronary artery disease in 12 (48%), abnormal ECG in 18 (72%), relevant conduction system disease in 9 (36%), restriction / constriction in 7 (28%), and pericardial effusion in 4 (16%). Cardiac surgery was necessary in 12 patients (death in 2 patients), percutaneous coronary interventions in 5 and pacemaker implantation in 3. Endocarditis occurred in 2 patients. During follow-up (21 ± 8 months), death occurred in 6 patients (24%) and was due to heart disease in 5 of them. Conclusions: If symptomatic heart disease develops in the long-term follow-up after RT, complex disease of valves, coronary arteries, conduction system, myocardium and pericardium is frequently observed. Cardiac interventions are often necessary; and heart disdisease may be a common cause of death in these patients. Careful assessment and evaluation of treatment options are needed in this patient group

Long-term follow-up, computed tomography, and computational fluid dynamics of the Cabrol procedure

OBJECTIVES: The Cabrol procedure is characterized by insertion of an ascending aortic composite graft with reimplantation of the coronary arteries by the interposition of a graft tube. Our purpose is to report the clinical long-term follow-up and computed tomographic findings in patients having undergone the Cabrol procedure and to determine blood flow in the Cabrol graft using computational fluid dynamics. METHODS: Clinical follow-up (76.6 +/- 16.6 months) and dual-source computed tomographic angiography data of 7 patients (all men, mean age 54.9 +/- 9.6 years) with 12 Cabrol grafts (left main coronary artery, n = 7; right coronary artery, n = 5) were reviewed. In 2 patients, the right coronary artery was directly reattached to the aortic graft. Computational fluid dynamics were calculated using computed tomographic data of a patient with the Cabrol procedure and compared with those in a Valsalva graft and a healthy aortic root. RESULTS: Computed tomography showed Cabrol graft occlusions to 1 of 7 (14%) left main and of 2 of 5 (40%) right coronary arteries. Six grafts to the left main and 3 to the right coronary artery were fully patent, similar to the 2 directly reattached right coronary arteries to the aortic graft. Computational fluid dynamics results show similar blood flow parameters into the coronaries for the healthy aortic root and Valsalva graft. In the Cabrol graft, a spiraling flow pattern with low flow into the right coronary artery was found (right coronary artery = 1 mL/min at both systole and diastole). CONCLUSIONS: Our study indicates low flow rates particularly in the right Cabrol graft correlating with a higher incidence of occlusions of the right as compared with the left Cabrol graft at long-term follow-up

Remodelling after surgical repair of atrial septal defects within the oval fossa

In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 +/- 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 +/- 18 years, and length of follow-up was up to 40 years (11 +/- 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 +/- 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias