Why do we need randomized and epidemiological studies on cardiovascular disease? Evidence-based cardiology VII

Over the last two decades the results of randomized clinical studies, which are powerful aids for correctly assessing therapeutical strategies, have consolidated cardiological practice. In addition, scientifically interesting hypotheses have been generated through the results of epidemiological studies. Properly conducted randomized studies without systematic errors and with statistical power adequate for demonstrating moderate and reasonable benefits in relevant clinical outcomes have provided reliable and strong results altering clinical practice, thus providing adequate treatment for patients with cardiovascular disease (CVD). The dissemination and use of evidence-based medicine in treating coronary artery disease (CAD), heart failure (HF), and in prevention will prevent hundreds of thousands of deaths annually in developed and developing countries. CVD is responsible for approximately 12 million deaths annually throughout the world, and approximately 60% of these deaths occur in developing countries. During recent years, an increase in mortality and morbidity rates due to CVD has occurred in developing countries. This increase is an indication that an epidemiological (demographic, economical, and health-related) transition is taking place in developing countries and this transition implies a global epidemic of CVD, which will require wide-ranging and globally effective strategies for prevention. The identification of conventional and emerging risk factors for CVD, as well as their management in high-risk individuals, has contributed to the decrease in the mortality rate due to CVD. Through a national collaboration, several multi-center and multinational randomized and epidemiological studies have been carried out throughout Brazil, thus contributing not only to a generalized scientific growth in different Brazilian hospitals but also to the consolidation of an increasingly evidence-based clinical practice.

Anomalous origin of the left coronary artery from the pulmonary trunk. Clinical features and midterm results after surgical treatment

OBJECTIVE: To report the authors' experience with the anomalous origin of the left coronary artery (AOLCA) from the pulmonary trunk, emphasizing preoperative data, surgical aspects and midterm results of the follow-up. METHODS: Retrospective analysis of 11 patients operated upon at the Royal Brompton Hospital from October, 84 to April, 97. RESULTS: Nine infants had heart failure (HF) and two other children presented with dyspnea and chest pain. All had ECG changes. The echocardiogram identified the anomalous origin of the coronary artery in 7 (64%) patients and hemodynamic studies were performed in 7 patients. All infants were operated upon between the 2nd and 10th month of life. Six patients were treated with aortic reimplantation of the left coronary artery, whereas five were operated upon according to the Takeuchi technique. All patients are alive, with clear improvement of the ECG changes and ventricular function, as evaluated by echocardiography. Two patients operated upon according to the Takeuchi technique required additional surgery due to severe supravalvular pulmonary stenosis. CONCLUSION: AOLCA is a rare disease. Most patients show early signs of severe HF associated with ECG findings. Surgical therapy must be instituted early in the disease, preferentially through aortic implantation of the anomalous coronary artery, with a high possibility of success. Shortly after surgery, clinical and ECG improvement, as well as normalization of left ventricular function, should be expected.

Atrial infarction is a unique and often unrecognized clinical entity

A patient with heart failure and acute atrial fibrillation received the final diagnosis of atrial infarction associated with ventricular infarction based on clinical findings of ischemia in association with atrial fibrillation and heart failure (mechanisms probably involved: contractile dysfunction and loss of atrial contribution). Although a transesophageal echocardiography, which could refine the diagnosis of anatomic abnormalities, was not performed, all evidence led to the diagnosis of atrial involvement. Electrocardiographic findings were consistent with Liu's major criterion 3. Therapy with digitalis, quinidine and angiotensin-converting enzyme inhibitors was chosen, as the patient had acute pulmonary edema. The use of beta-blockers and verapamil was restricted. No other complications, such as thrombo-embolism or atrial rupture, were noted.

Rheumatic carditis treated with high doses of pulsetherapy methylprednisolone. Results in 70 children over 12 years

PURPOSE: To report the result of patients treated with IV methylprednisolone divided into three groups and compare their follow-up during the last 12 years. METHODS: Seventy children with active rheumatic carditis (76 episodes) in heart failure Class III and IV (NYHA) were studied. The diagnosis was based on modified Jones' criteria. After ruling out infections and strongyloidiasis, treatment with IV methylprednisolone bolus was started three times a week until the laboratory tests became negative. Patients were divided into 3 groups, according to the time of hospital admittance: Groups 1, 2 and 3, comprising of 40, 18 and 12 children, respectively. RESULTS: Eighteen children in Group 1 (45%) were in their 1st attack: 2 series of pulsetherapy were used in 10 (25%), 3 in 9 (23%) and 4 in 21 (52%). In Group 2, 14 cases (77%) were in their 1st attack: 2 series were used in 7 (39%), 4 in 9 (50%) and 5 in 2 (11%). The echocardiogram showed a flail mitral valve in 12 (66%) of these patients (1 death occurred after mitral valvoplasty). In Group 3, 6 patients needed 5 or more series of pulsetherapy and a flail mitral valve was present in 5 (41%). One child underwent mitral valve replacement while still in the active phase, after 8 series of pulsetherapy, and another died. The number of patients who needed 5 or more series was significantly higher in Group 3. CONCLUSION: There were variations in the presentation and evolution of the cases during these 12 year. The established pulsetherapy protocol continues to be useful to treat severe cases.

Myxoma of the mitral valve

Only rarely do myxomas originate from the mitral valve. This is the report of a 49-year-old woman presenting with congestive heart failure. The diagnosis of an intracardiac tumor involving the anterior cuspid of the mitral valve was made by transesophageal echocardiography. The patient underwent surgery for tumor resection and plasty of the valve was made with reconstruction and preservation of the valve. The diagnosis of myxoma was confirmed by histology. This is the 23rd case of myxoma of the mitral valve reported in the literature.

Natural course of subsequent pregnancy after peripartum cardiomyopathy

OBJECTIVE: To assess the effect of subsequent pregnancy after peripartum cardiomyopathy (PPCM) on maternal and fetal outcome. METHODS: Prospective study of 34 patients with the diagnosis of PPCM (mean age= 26years). At the time of first diagnosis 5 were in NYHA functional class (FC) II for heart failure, one in FC III and 28 in FC IV. After clinical treatment, patients were advised to avoid new pregnancies and a follow-up was obtained. RESULTS: There were 12 (35.3%) subsequent pregnancies in patients (pt) aged 19 to 44 years (mean 32), divided into two groups: GI: 6 pts who had normalized their heart size and GII: 6 pts with persistent cardiomegaly. GI had initially mild clinical manifestations ( 3 were in FC II, 1 in FC II and 2 in FC IV) and complete recovery of cardiac function (FC I). A new pregnancy was well-tolerated in 5 (83.3%); 1 pt presented with preeclampsia, and progressed to FC II. Presently, 5 pt are in FC I and 1 in FC II. GII pts had more severe heart failure at the onset of PPCM (1 pt in FC II and 5 in FC IV); during follow-up, 4 pt were in FC I and 2 in FC II. A new pregnancy was well tolerated in all of them, but the eldest, who had had 2 pregnancies and had a progressive worsening of clinical status, dying 8 years after the last pregnancy and 13 years after the diagnosis of PPCM. The remaining 5 pt are still alive, 3 in FC I and 2 in FC II, with worsening of FC in 1. Subsequent pregnancies occurred 3-7 years after clinical treatment of PPCM and no fetal distress was observed. CONCLUSION: Subsequent pregnancies are well-tolerated after PPCM, but not devoid of risk. No fetal distress was observed. A minimum interval of 3 years after the recovery of function seems to be safe for subsequent pregnancies.

Aortopulmonary window. Clinical and surgical assessment of 18 cases

OBJECTIVE: Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS: Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS: Heart failure occurred in 14 patients, and cyanosis in 3: 2 from GB (tetralogy of Fallot - TF, and double outlet right ventricle - DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION: APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.

Anomalous origin of the left coronary artery from the right pulmonary artery with intramural aortic trajectory. Clinicosurgical diagnostic implications

OBJECTIVE: Anomalous origin of the left coronary artery from the right pulmonary artery (AOLCARPA), is a rare entity that is usually associated with other defects. Of the 20 cases of AOLCARPA reported in the literature, 14 (70%) had associations. We describe four patients with AOLCARPA without associated defects, but with a peculiar intramural aortic trajectory. METHODS: Fifty-five patients with anomalous origin of the left coronary artery were operated upon at INCOR-FMUSP. Four of the patients had the anomalous origin from the right pulmonary artery (RPA) without associated defects but with intramural aortic trajectory. Clinical and laboratory examinations were analyzed, as well as surgical findings. RESULTS: All patients had congestive heart failure (CHF) and 3 also had angina pectoris. Two patients had a murmur of mitral regurgitation, signs of myocardial infarction on the ECG and cardiomegaly. The shortening fraction varied from 9% to 23%. The hemodynamic study confirmed the diagnosis of anomalous origin of the coronary artery, but the intramural trajectory and the origin from the RPA were established only at surgery. In 3 patients, the technique of side-to-side anastomosis was performed with a good outcome. One patient, who underwent end-to-side anastomosis, died 6 months after the surgery. CONCLUSION: Association with other defects usually occurs in the AOLCARPA, and the intramural aortic trajectory is difficult to clinically diagnose but easy to surgically correct.

Ebstein's anomaly with coarctation of the aorta. An unusual association

Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solitus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a DeVega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.

Fatal pulmonary embolism in hospitalized patients. Clinical diagnosis versus pathological confirmation

OBJECTIVE - To assess the incidence of fatal pulmonary embolism (FPE), the accuracy of clinical diagnosis, and the profile of patients who suffered an FPE in a tertiary University Hospital. METHODS - Analysis of the records of 3,890 autopsies performed at the Department of General Pathology from January 1980 to December 1990. RESULTS - Among the 3,980 autopsies, 109 were cases of clinically suspected FPE; of these, 28 cases of FPE were confirmed. FPE accounted for 114 deaths, with clinical suspicion in 28 cases. The incidence of FPE was 2.86%. No difference in sex distribution was noted. Patients in the 6th decade of life were most affected. The following conditions were more commonly related to FPE: neoplasias (20%) and heart failure (18.5%). The conditions most commonly misdiagnosed as FPE were pulmonary edema (16%), pneumonia (15%) and myocardial infarction (10%). The clinical diagnosis of FPE showed a sensitivity of 25.6%, a specificity of 97.9%, and an accuracy of 95.6%. CONCLUSION - The diagnosis of pulmonary embolism made on clinical grounds still has considerable limitations.

Pompe's disease or type IIa glycogenosis

This is the report of a five-month-old child presenting clinical evidence of Pompe's disease: severe hypotonicity, hyporeflexia and congestive heart failure. The ECG showed a short PR interval, the chest radiography disclosed marked cardiomegaly, and the echocardiogram revealed marked left ventricular hypertrophy - the most typical finding of this disease. A skeletal muscle biopsy led to final diagnosis, because in the histopathologic study marked increased glycogen accumulation was evident. Death occurred two months after symptom onset.

Endomyocardial fibrosis associated with massive calcification of the left ventricle

This is the report of a rare case of endomyocardial fibrosis associated with massive calcification of the left ventricle in a male patient with dyspnea on great exertion, which began 5 years earlier and rapidly evolved. Due to lack of information and the absence of clinical signs that could characterize impairment of other organs, the case was initially managed as a disease with a pulmonary origin. With the evolution of the disease and in the presence of radiological images of heterogeneous opacification in the projection of the left ventricle, the diagnostic hypothesis of endomyocardial disease was established. This hypothesis was later confirmed on chest computed tomography. The patient died on the 16th day of the hospital stay, probably because of lack of myocardial reserve, with clinical findings of refractory heart failure, possibly aggravated by pulmonary infection. This shows that a rare disease such as endomyocardial fibrosis associated with massive calcification of the left ventricle may be suspected on a simple chest X-ray and confirmed by computed tomography.

A new surgical approach for treating dilated cardiomyopathy with mitral regurgitation

OBJECTIVE: To evaluate the early outcome of mitral valve prostheses implantation and left ventricular remodeling in 23 patients with end-stage cardiomyopathy and secondary mitral regurgitation (NYHA class III and IV). METHODS: Mitral valvular prosthesis implantation with preservation of papillary muscles and chordae tendinae, and plasty of anteriun cuspid for remodeling of the left ventricle. RESULTS: The surgery was performed in 23 patients, preoperative ejection fraction (echocardiography) varied from 13% to 44% (median: 30%). In 13 patients associated procedures were performed: myocardial revascularization (9), left ventricle plicature repair (3) and aortic prosthese implantation (1). Early deaths (2) occurred on the 4th PO day (cardiogenic shock) and on the 20th PO day (upper gastrointestinal bleeding), and a late death in the second month PO (ventricular arrhythmia). Improvement occurred in NYHA class in 82.6% of the patients (P<0.0001), with a survival rate of 86.9% (mean of 8.9 months of follow-up). CONCLUSION: This technique offers a promising therapeutic alternative for the treatment of patients in refractory heart failure with cardiomyopathy and secondary mitral regurgitation.

Peripheral arterial embolism. Report of hospitalized cases

OBJECTIVE: We analyzed the frequency of peripheral embolisms, the underlying heart disease,triggering factors, the sites of the emboli, and evolution of the patients. METHODS: We analyzed 29 cases of peripheral arterial embolism out of a total of 20,211 hospitalizations in a cardiology center in the city of São Paulo. The age was 51.89±18.66 years, and 15 were males. RESULTS: Embolism in the right lower limb occurred in 18 patients (62.0%),in the left lower 11(37.9%) and right upper 3 (10.3%) limbs, and in the left arm (1). Four patients had embolism in two limbs. The heart disease, mitral valvar heart disease (9 patients - 31.0%); infective endocarditis (7- 24.1%); dilated cardiomyopathy (6 - 20.6%); ischemic coronary heart disease (6 patients - 20.6%); and one patient with cor pulmonale. Atrial fibrillation was observed in 20 patients (68.9%), chronic in 12 patients (41.3% ) and acute in 8 (27.5%). All patients with mitral valvar heart disease had atrial fibrillation, chronic in 8 patients (88.8%); patients with cardiomyopathy and coronary heart disease, 4 in each group had atrial fibrillation, acute in 60% of the patients.Patients with infective endocarditis, 3 had staphylococcus and 2 Gram-negative bacteria. In the follow-up, 2 patients (6.8%) required limbs amputation, and 5 (17.2%) died due to embolism. CONCLUSION: Most of the time, embolism does not cause permanent complications. Our data highlight the importance of anticoagulation for patients acute atrial fibrillation in myocardial dysfunction and for patients with chronic atrial fibrillation in cases of mitral valvar heart disease to prevent peripheral embolism.

Prognostic factors of rheumatic mitral stenosis during pregnancy and puerperium

OBJECTIVE: To identifity characteristics associated with complications during pregnancy and puerperium in patients with rheumatic mitral stenosis. METHODS: Forty-one pregnant women (forty-five pregnancies) with mitral stenosis, followed-up from 1991 to 1999 were retrospectively evaluated. Predictor variables: the mitral valve area (MVA), measured by echocardiogram, and functional class (FC) before pregnancy (NYHA criteria).Maternal events: progression of heart failure, need for cardiac surgery or balloon mitral valvulotomy, death, and thromboembolism. Fetal/neonatal events: abortion, fetal or neonatal death, prematurity or low birth weight (<2,500g), and extended stay in the nursery or hospitalization in newborn ICU. RESULTS: The mean ± SD of age of the patients was 28.8±4.6 years. The eventful and uneventful patients were similar in age and percentage of first pregnancies. As compared with the level 1 MVA, the relative risk (RR) of maternal events was 5.5 (95% confidence interval (CI) =0.8-39.7) for level 2 MVA and 11.4 (95% CI=1.7-74.5) for level 3 MVA. The prepregnancy FC (FC > or = II and III versus I) was also associated with a risk for maternal events (RR=2.7; 95% CI=1.4-5.3).MVA and FC were not importantly associated with these events, although a smaller frequency of fetal/neonatal events was observed in patients who had undergone balloon valvulotomy. CONCLUSION: In pregnant women with mitral stenosis, the MVA and the FC are strongly associated with maternal complications but are not associated with fetal/neonatal events. Balloon mitral valvulotomy could have contributed to reducing the risks of fetal/neonatal events in the more symptomatic patients who had to undergo this procedure during pregnancy.