Multi atrial maco-re-entry circuits in adults with repaired congenital heart disease: entrainment mapping combined with three-dimensional electroanatomic mapping.

OBJECTIVES: We sought to characterize re-entry circuits causing intra-atrial re-entrant tachycardias (IARTs) late after the repair of congenital heart disease (CHD) and to define an approach for mapping and ablation, combining anatomy, activation sequence data and entrainment mapping. BACKGROUND: The development of IARTs after repair of CHD is difficult to manage and ablate due to complex anatomy, variable re-entry circuit locations and the frequent co-existence of multiple circuits. METHODS: Forty-seven re-entry circuits were mapped in 20 patients with recurrent IARTs refractory to medical therapy. In the first group (n = 7), ablation was guided by entrainment mapping. In the second group (n = 13), entrainment mapping was combined with a three-dimensional electroanatomic mapping system to precisely localize the scar-related boundaries of re-entry circuits and to reconstruct the activation pattern. RESULTS: Three types of right atrial macro-re-entrant circuits were identified: those related to a lateral right atriotomy scar (19 IARTs), the Eustachian isthmus (18 IARTs) or an atrial septal patch (8 IARTs). Two IARTs originated in the left atrium. Radiofrequency (RF) lesions were applied to transect critical isthmuses in the right atrium. In three patients, the combined mapping approach identified a narrow isthmuses in the lateral atrium, where the first RF lesion interrupted the circuit; the remaining circuits were interrupted by a series of RF lesions across a broader path. Overall, 38 (81%) of 47 IARTs were successfully ablated. During follow-up ranging from 3 to 46 months, 16 (80%) of 20 patients remained free of recurrence. Success was similar in the first 7 (group 1) and last 13 patients (group 2), but fluoroscopy time decreased from 60 +/- 30 to 24 +/- 9 min/procedure, probably related to the increasing experience and ability to monitor catheter position non-fluoroscopically. CONCLUSIONS: Entrainment mapping combined with three-dimensional electroanatomic mapping allows delineation of complex re-entry circuits and critical isthmuses as targets for ablation. Radiofrequency catheter ablation is a reasonable option for treatment of IARTs related to repair of CHD.

Multiple floppy valves: an echocardiographic syndrome.

Echocardiograms of 400 patients with mitral valve prolapse examined at the Peter Bent Brigham Hospital between 1974 and 1977 were reviewed. Eleven patients (3 per cent) were found to have prolapse (10 patients) or large excursion of the tricuspid valve (one patient) and large excursion of the aotric valve (four patients) or dilatation of the aotric root (seven patients) in addition to mitral valve prolapse. Two of these 11 patients underwent mitral valve replacement, and myxomatous degeneration of the valves was noted on pathologic examination. Almost half of the patients with multiple floppy valves (five of 11) had symptoms of congestive heart failure. In contrast to reported series of isolated mitral valve prolapse, in which female preponderance has been documented, 10 of the 11 patients were male. The syndrome of multiple floppy valves may represent either a unique entity or a more advanced form of the same process which underlies mitral valve prolapse.

Valvular heart disease (aortic regurgitation and mitral valve prolapse) among institutionalized adults with Down's syndrome.

Congenital heart disease has long been recognized in children with Down's syndrome, but little is known about the manifestations of clinical heart disease in adults with this condition. Therefore, 131 adults with Down's syndrome were examined. Clinical heart disease was considered to be present when an abnormally split S2, systolic click, at least grade 3/6 systolic precordial murmur, or any diastolic precordial murmur was heard. Using these criteria, 38 patients had clinical heart disease and 93 did not. To confirm and evaluate the auscultatory findings, echocardiograms were recorded in 37 of the patients. Comparison between patients without clinical heart disease and those with clinical heart disease showed that neither age (39 +/- 11 vs 40 +/- 12 years, respectively) nor gender (60% vs 66% men, respectively) differed significantly. Eleven had clinical and echocardiographic findings consistent with atrial or ventricular septal defect. Findings consistent with aortic regurgitation were identified in 8, and 18 had mitral valve prolapse. These results suggest that in addition to atrial and ventricular septal defect (which have a well recognized association with Down's syndrome), 2 specific, usually asymptomatic, and heretofore unanticipated valvular cardiac abnormalities may be associated with Down's syndrome.

Value of a noninvasively induced ventricular extrasystole during echocardiographic and phonocardiographic assessment of patients with idiopathic hypertrophic subaortic stenosis.

Fifteen patients with idiopathic hypertrophic subaortic stenosis had a ventricular extrasystole induced with a new external mechanical cardiac stimulator during noninvasive evaluation of left ventricular outflow tract obstruction. Ten patients were monitored with simultaneous echocardiogram, phonocardiogram and indirect carotid pulse tracing; five were monitored with the phonocardiogram and indirect carotid pulse tracing alone. Nine of the 15 patients showed obstruction in the beat after the ventricular extrasystole, as defined by appearance of the characteristic bifid carotid pulse contour and, where recorded, an increase in systolic anterior motion of the mitral valve on echocardiography. Six patients did not show obstruction. All nine patients with obstruction had greater than 20 msec prolongation of uncorrected systolic ejection time in the post-extrasystolic beat of the carotid pulse tracing. Change in the uncorrected ejection time was + 0.038 +/- 0.15 second (mean +/- standard deviation) in these nine patients compared with -0.003 +/- 0.005 second in the six not showing obstruction (P less than 0.01). Six patients underwent cardiac catheterization: Three patients without obstruction after a noninvasively induced ventricular extrasystole had no obstruction at catheterization and three patients with obstruction after noninvasively induced ventricular premature beats demonstrated obstruction at rest or after provocative maneuvers during catheterization. These results indicate that the noninvasive induction of a ventricular extrasystole is a useful and easily performed procedure for both diagnosing and evaluating the dynamic left ventricular outflow tract obstruction of idiopathic hypertrophic subaortic stenosis.

Administration of nitrous oxide in normal subjects. Evaluation of systems of gas delivery for their clinical use and hemodynamic effects.

Nitrous oxide (concentrations of 30 to 50%) was administered to 22 healthy volunteer subjects via nasal prongs, rebreathing mask, and an airlines mask to assess the efficiency of systems of delivery and the hemodynamic effects. The end-expired concentration of nitrous oxide, expressed as a percentage of the inspired concentration of nitrous oxide, was 19% for nasal prongs, 34% for the rebreathing mask, and 95% for the airlines mask (most accurate delivery). The pulse rate fell from a mean of 79+/-3 beats per minute to 67+/-2 with nasal prongs, to 64+/-2 with the rebreathing mask, and to 64+/-2 with the airlines mask. Both systolic and diastolic pressures fell from means of 122+/-4 and 74+/-2 mm Hg, respectively, to 98+/-3 mm Hg and 64+/-2 mm Hg, respectively, with the airlines apparatus. The fall in systolic blood pressure (slope, -0.79) exceeded that in the diastolic (slope, -0.35). In additional eight normal subjects, administration of 30% nitrous oxide via airlines mask produced identical changes in blood pressure and pulse rate, but there was no effect from 30 minutes of administration of 30% nitrous oxide on the end-systolic volume index, cardiac index, ejection fraction, normalized wall velocity determined echocardiographically, ejection time, or the ratio of preejection period to ejection time.

Aortic and mitral insufficiency complicating fulminant systemic lupus erythematosus.

The cardiac complications of systemic lupus erythematosus (SLE) include a multitude of valvular, myocardial, and pericardial abnormalities resulting from acute and chronic inflammation involving the endocardium, myocardium, and/or pericardium. A case of acute, severe, aortic, and mitral insufficiency occurring as discrete complications of consecutive flares of SLE in the same patient is described with particular emphasis on the clinical and gross pathologic findings. The cardiac complications of SLE, both from a pathologic and clinical standpoint, are reviewed in the context of the uniqueness of this case.

Conservative management of uremic pericardial effusions.

Although there has been a recent trend toward early operative treatment of uremic pericardial effusions unresponsive to intensified dialysis, this approach may be unnecessarily aggressive. Review of 787 patients in our chronic dialysis program since 1969 has shown 54 patients (6.9 percent) to have developed 56 episodes of large pericardial effusion. All were managed by increasing the frequency of dialysis. If the effusion failed to diminish or if life-threatening signs of tamponade developed, pericardiocentesis was performed. In 63 percent (35/56) the effusion resolved with increased dialysis. In 37 percent (21/56), pericardiocentesis was performed, with 57 percent (12/21) requiring only one aspiration. During a mean follow-up of 34 months (2 to 100 months) only 5.5 percent (3/54) have undergone operation: one partial pericardiectomy incidental to pulmonary decortication and two pericardiectomies for late (3 months and 5 months, respectively) constriction. There were five complications of pericardiocentesis: one pneumothorax, one pneumoperitoneum, one costochondritis, and two myocardial punctures without sequelae. The one death related to pericardial effusion in this series occurred in a home-dialysis patient who arrived in the emergency room moribund. Our experience suggests that the great majority of uremic pericardial effusions can be effectively controlled with simple needle aspiration by experienced personnel and that pericardial resection is usually not necessary.

Women's health education in rural Bangladesh.

Each year in Bangladesh scores of women and children suffer and die from diseases that are largely preventable or curable. In an effort to address this problem, CARE-Bangladesh established its Women's Health Education (WHE) program. The WHE program, which targets poor women in rural areas, was designed to teach women how to prevent and treat health problems which routinely afflict them and their families. The health education sessions emphasize preventative measures and cover topics concerning health and hygiene of women and children. Surveys are conducted immediately following course completion and again six months later to assess the short and long-term impact of the program. Surveys conducted at the end of the 12 week course period indicate that, on average, participants retain 97% of the material covered. Measurement of knowledge retention 6 months after course completion revealed only a 5% decline from the post-course survey results. These findings illustrate the program's success in increasing knowledge and awareness of health interventions. Since imparting knowledge is a first step toward changing behavior, WHE's success in this regard suggests a significant role for health education in preventing and treating common health hazards, and thus in improving the health status of women and children in developing countries.

Fistulous aneurysm of ductus arteriosus.

We present clinical, haemodynamic, and angiographic data on a 48-year-old woman with a fistulous aneurysm of the ductus arteriosus. This is the first reported case of a spontaneous fistulous aneurysm of the ductus arteriosus in an adult in which an antemortem diagnosis was made. This lesion typically presents with shortness of breath, hoarseness caused by recurrent laryngeal nerve paralysis, and chest pain, and poses a critical threat to life because of progressive compression, erosion, and haemorrhage into the oesophagus or tracheobronchial tree. Fistulous aneurysm of the ductus arteriosus should be considered in the differential diagnosis of an enlarging mediastinal mass, especially when a continuous heart murmur is noted. Early definitive diagnosis and surgical repair in adults are essential for survival.

Heart transplantation for intractable prosthetic valve endocarditis.

A 25-year-old woman with active systemic lupus erythematosus and infective endocarditis was seen initially with porcine aortic bioprosthetic stenosis, perivalvar regurgitation, and native mitral regurgitation 9 years after aortic valve replacement for lupus endocarditis. Double-valve replacement was performed with St. Jude Medical mechanical prostheses. After operation the patient developed fever and an elevated white blood cell count. One month later she had increasing mitral and aortic perivalvular regurgitation and intermittent complete heart block. At reoperation both annuli showed evidence of continued infection, and she underwent annular reconstructions with pericardium and double-valve re-replacement. Cultures grew Mycoplasma hominis. Despite long-term therapy with appropriate antibiotics, within 2 months she developed recurrent perivalvar regurgitation with congestive heart failure. Orthotopic heart transplantation was performed. The postoperative course was notable for significant leukocytosis and spontaneous culture negative hemothorax that required thoracotomy for drainage. The patient recovered and is now well 14 months after operation.

Echocardiographic diagnosis of left atrial thrombus - a case report.

We report a case of mitral stenosis with echocardiographic findings we consider diagnostic of left atrial thrombus. Scanning of the ultrasound beam from the left ventricular basalward to the left atrium (LA) demonstrates a consistent group of strong echoes within the LA and extending into the mitral orifice. Massive left atrial thrombosis was confirmed at operation.

The diagnostic accuracy of combined clinical and noninvasive cardiac evaluation: comparison with findings at cardiac catheterization.

The accuracy of combined clinical and noninvasive cardiac diagnostic evaluation was prospectively examined in 108 consecutive patients referred to the heart station for echocardiographic examination prior to cardiac catheterization. History, physical examination, scalar electrocardiology, chest roentgenography, phonocardiography and pulse recording, and M-mode echocardiography were employed by the heart station cardiologist, who assigned one or more diagnoses to each patient. In addition, one of three management strategies was proposed for each patient: 1) surgery without cardiac catheterization; 2) medical therapy without cardiac catheterization; or 3) cardiac catheterization for clarification of the diagnosis. The results of the combined clinical and noninvasive evaluation were independently reviewed for each patient and compared with the diagnosis determined by cardiac catheterization, results of cardiac surgery, and total hospital course. Diagnostic predictions employing combined clinical and noninvasive cardiac evaluation were completely correct in 86% of patients, and management strategy was correct in 97% of individuals. In approximately one-half of all patients full cardiac catheterization or coronary arteriography was recommended. All management strategy errors and two-thirds of diagnostic errors occurred in patients with mitral regurgitation, aortic regurgitation, or coronary artery disease. Combined clinical and noninvasive evaluation results in accurate diagnostic information adequate for the formulation of appropriate management strategies in the majority of patients, but many individuals with cardiac disease still require invasive evaluation for complete diagnosis.

Serial studies of cardiac function in patients receiving adriamycin.

A total of 283 adult and pediatric patients treated with drug combinations which include adriamycin (ADM) had at least one cardiac evaluation; 103 patients had two or more serial studies after a baseline evaluation. Changes in systolic time intervals, echocardiographically determined ejection fractions, and electrocardiographic voltage correlated with increasing cumulative ADM dosage. However, the incidence of congestive heart failure among these patients was less than 2%. It is estimated that 22%-31% of these patients would not have completed a cumulative dose of greater than 450 mg/m2 of ADM if the drug had been discontinued on the basis of these tests, using previously published criteria for cessation of treatment. Thus far, however, no adverse effects have been observed from the completion of planned courses of therapy. The clinical significance of serial changes in noninvasive tests is therefore uncertain. While such changes may reflect subclinical anthracycline cardiotoxicity and clinical cardiotoxicity can reliably be diagnosed, the utility of routine, serial noninvasie testing in planning conventional-dose anthracycline therapy is questionable.

Noninvasive assessment of valvular heart disease: surgery without catheterization.

Forty-one patients underwent valve surgery at our institution based solely on clinical, M-mode echocardiographic, phonocardiographic, and external pulse recording findings without preoperative cardiac catheterization. Patients with clinical evidence of coronary artery disease were excluded from the study. Preoperatively, 83% of the patients were New York Heart Association functional class III or IV. In all patients, the noninvasive evaluation was considered sufficiently diagnostic of the nature and severity of valvular heart disease to allow surgery without preoperative catheterization. In 23 of 41 cases (group 1), cardiac catheterization was not performed due to the patients' unstable hemodynamic condition at the time surgery was being considered. In the remaining 18 patients (group 2), the probability of obtaining data at catheterization that would significantly affect management decisions was thought to be low, thus not justifying the cost and potential morbidity of this procedure. In all cases, the noninvasive diagnosis was corroborated at operation; there were no unexpected findings nor deaths related to incomplete or incorrect diagnoses. Over a followup period of 4.5 +/- 1.4 years, no patient experienced signs or symptoms of ischemic heart disease. In selected patients without anginal chest pain syndromes, appropriate and successful valve surgery may be performed on the basis of combined clinical and noninvasive evaluation without the need for cardiac catheterization.

Sick sinus syndrome after surgery for congenital heart disease.

The course and prognosis of 16 infants and children with sick sinus syndrome associated with cardiac surgery is reviewed. The dysrhythmia was observed most ofter after extensive atrial reconstructive surgery in patients with transposition of the great arteries and with atrial septal defect. In 12 (75%) of the patients, sick sinus syndrome was detected in the immediate postoperative period. Tachyarrhythmias and bradyarrhythmias were presnet in 12 and isolated bradyarrhythmias in four. Temporary pacing was used in two and permanent pacing was required in five. Death in two non-paced patients was attributable to arrhythmias. Postmortem examination in one patient revealed a suture in the sinoatrial node. Careful attention to the anatomy of the sinoatrial node, its artery and the internodal tracts during surgery may prevent the development of sick sinus syndrome. In patients with dysrhythmia, a careful search should be undertaken to document the abnormal rhythm with the use of Holter monitoring. The insertion of a pacemaker is indicated in patients with tachyarrhythmias requiring cardioversion or antiarrhythmic drug therapy and those without adequate lower escape mechanisms.

Tricuspid stenosis masquerading as right ventricular failure in a patient with a prosthetic mitral valve.

Echocardiographic, phonocardiographic, and pulse tracing recordings demonstrated clinically unsuspected tricuspid stenosis in a 53-year-old man with a prosthetic mitral valve and signs of right heart failure. Physiologic correlates of the patient's heart sounds and diastolic murmur were studied by simultaneous M-mode echocardiograms and phonocardiograms. Cardiac catheterization and subsequent valve surgery confirmed the presence of tricuspid stenosis. It is important to consider the diagnosis of tricuspid stenosis in all patients with a history of rheumatic mitral valve disease who develop findings suggestive of right ventricular failure.