Calcific retinal embolism as an indicator of severe unrecognised cardiovascular disease.

OBJECTIVE: To describe the association between calcific retinal embolism (CRE) and cardiac valve stenosis. DESIGN AND SETTING: Retrospective chart review of patients with clinical criteria for CRE. PATIENTS: 24 patients with CRE who underwent two dimensional echocardiography between 1976 and 1998. RESULTS: Nine patients (38%) had calcific valve stenosis, which was haemodynamically severe in five patients (four aortic and one mitral), four of whom had no cardiac symptoms. Six patients underwent surgical intervention (aortic valve replacement in three patients, mitral and aortic valve replacement in one patient, removal of calcific cardiac pseudotumour in one patient, and carotid endarterectomy in one patient). CONCLUSIONS: CRE may be the presenting feature of otherwise asymptomatic, clinically important underlying cardiovascular disease and, in particular, haemodynamically severe calcific valve stenosis.

Ebstein's anomaly - review of a multifaceted congenital cardiac condition.

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Management of EA patients is complex. Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease.

Coarctation of the aorta: outcome of pregnancy.

OBJECTIVES: The study sought to determine the outcome of pregnancy in women with coarctation of the thoracic aorta. BACKGROUND: Patients with coarctation of the thoracic aorta are expected to reach childbearing age, but data on the outcome of pregnancy in this population are limited. METHODS: The Mayo Clinic database was reviewed for women of childbearing age (> or =16 years old) with a diagnosis of aortic coarctation evaluated from 1980 to 2000. Spectrum of cardiovascular disease, surgical history, and obstetrical and neonatal outcomes were determined. RESULTS: Fifty women with coarctation had pregnancies: 30 had coarctation repair before pregnancy, 10 had repair after pregnancy, 4 had repair both before and after pregnancy, and 6 had no history of repair. The 50 women had 118 pregnancies resulting in 106 births. There were 11 miscarriages (9%), 4 premature deliveries (3%), and 1 early neonatal death; 38 deliveries (36%) were by cesarean section. Of the 109 offspring, 4 (4%) had congenital heart disease. A patient with Turner syndrome died of a Stanford type A dissection at 36 weeks of pregnancy. Nineteen women (38%) were known to have hemodynamically significant coarctation during pregnancy (gradient > or =20 mm Hg). Fifteen women (30%) had hypertension during their pregnancy, 11 of whom (73%) had hemodynamically significant coarctation during that time (8 with native and 3 with residual/recurrent coarctation). CONCLUSIONS: Major cardiovascular complications were infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Systemic hypertension during pregnancy was common and related to the presence of a significant coarctation gradient.

Operation for anorexigen-associated valvular heart disease.

OBJECTIVES: Recently, valvular regurgitation has been observed in patients who have taken fenfluramine or dexfenfluramine with or without phentermine. This study describes the clinical, echocardiographic, and pathologic findings of anorexigen-associated valvular heart disease and the surgical interventions required to treat it. METHODS: We reviewed clinical information on 14 patients with severe anorexigen-associated valvular disease who underwent cardiac operations. RESULTS: Thirteen women (mean age 44.2 +/- 5.3 years) received fenfluramine, 58.5 +/- 22.3 mg/day, and phentermine, 32.1 +/- 11.4 mg/day, for an average of 12.1 +/- 7.3 months before presentation. One woman received dexfenfluramine, 30 mg/day for 13 months, and phentermine, 60 mg/day, concomitantly for 6 months. Presenting symptoms included dyspnea (12 cases), palpitations (3), and atypical chest pain (3). Six patients had heart failure, and 4 had a new murmur. Echocardiography demonstrated severe mitral valve regurgitation in all patients. Seven also had aortic regurgitation, and 4 had significant tricuspid regurgitation. Four patients had successful mitral valve repair, 1 with concomitant aortic valve repair. Ten additional patients eventually required mitral valve replacement, 5 with concomitant aortic valve replacement. Excised valves demonstrated a glistening white appearance with plaque-like encasement of leaflets and chordae. Focal surface proliferation and fibrosis with a "stuck-on" appearance was consistently found. CONCLUSIONS: Anorexigen use may lead to severe multivalvular regurgitation with characteristic echocardiographic and pathologic findings. Recognition of drug-induced valvulopathy is important because of widespread use of these medications and the uncertain natural history of the disease. Early surgical experience suggests that valve repair is possible in these young patients.

Posterior pericardial ascending-to-descending aortic bypass: an alternative surgical approach for complex coarctation of the aorta.

BACKGROUND: Coarctation of the aorta is commonly associated with recoarctation or additional cardiovascular disorders that require intervention. The best surgical approach in such patients is uncertain. Ascending-to-descending aortic bypass graft via the posterior pericardium (CoA bypass) allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation. METHODS AND RESULTS: Between 1985 and 2000, 18 patients (13 males and 5 females, mean age 43+/-13 years) with coarctation of the aorta underwent CoA bypass through median sternotomy. Before operation, average New York Heart Association class was II (range I to IV), and 15 patients (83%) had systemic hypertension. One or more previous cardiovascular operations had been performed in 12 patients (67%); 10 patients had >/=1 prior coarctation repair. Two patients had prior noncoarctation cardiovascular surgery. Concomitant procedures performed in 14 patients (78%) included the following: aortic valve replacement in 9; coronary artery bypass surgery in 3; mitral valve repair in 2; and septal myectomy, mitral valve replacement, aortoplasty, subaortic stenosis resection, ventricular septal defect closure, and ascending aorta replacement in 1 patient each. All patients survived the operation and were alive with patent CoA bypass at a mean follow-up of 45 months. No graft-related complications occurred, and there were no instances of stroke or paraplegia. Systolic blood pressure fell from 159 mm Hg before surgery to 125 mm Hg after surgery. CONCLUSIONS: CoA bypass via median sternotomy can be performed with low morbidity and mortality. Although management must be individualized, extra-anatomic CoA bypass via the posterior pericardium is an excellent single-stage approach for patients with complex coarctation or recoarctation and concomitant cardiovascular disorders.

Impact of intraoperative transesophageal echocardiography among patients undergoing aortic valve replacement for aortic stenosis.

In this study, we sought to define the impact of intraoperative transesophageal echocardiography (IOTEE) among patients undergoing aortic valve replacement for severe aortic stenosis. We reviewed the clinical data and preoperative, intraoperative, and postoperative echocardiograms of all adults who underwent aortic valve replacement for aortic stenosis and had IOTEE between January 1993 and December 1996. There were 383 patients (223 men, 160 women; mean age, 69 years). Fifty-four (14%) of the 383 patients had mitral valve surgery at the time of aortic valve replacement. In 6 patients, mitral valve surgery was not planned but was added because of findings on IOTEE. In 25 patients, mitral valve surgery was canceled on the basis of the IOTEE. Additional information was found by IOTEE in 25 patients before and after bypass, altering the surgical plan in 18 of these 25 patients. Overall, IOTEE altered the planned operation in 49 (13%) of the 383 patients. These data support the routine use of IOTEE among patients undergoing aortic valve replacement for aortic stenosis.

Surgical management of left-sided carcinoid heart disease.

BACKGROUND: Carcinoid involvement of left-sided heart valves has been reported in patients with a patent foramen ovale, carcinoid tumor of the lung, and active carcinoid syndrome with high levels of serotonin. The present study details the clinical features and surgical management of patients with carcinoid heart disease affecting both left- and right-sided valves. METHODS AND RESULTS: Eleven patients (7 men, 4 women) with symptomatic carcinoid heart disease underwent surgery for left- and right-sided valve disease between 1989 and 1999. Mean age was 57+/-9 years, and median preoperative NYHA class was 3. All patients had metastatic carcinoid tumors and were on somatostatin analog. Of 11 patients, 5 (45%) had a patent foramen ovale; 1 of these also had a primary lung carcinoid tumor. Surgery included tricuspid valve replacement in all patients, pulmonary valve replacement in 3 and valvectomy in 7, mitral valve replacement in 6 and repair in 1, aortic valve replacement in 4 and repair in 2, CABG in 2, and patent foramen ovale closure in 5. One myocardial metastatic carcinoid tumor was removed. There were 2 perioperative deaths. At a mean follow-up of 41 months, 4 additional patients were dead. All but 1 surgical survivor initially improved >/=1 functional class. No patient required reoperation. CONCLUSIONS: Carcinoid heart disease may affect left- and right-sided valves and occurred without intracardiac shunting in 55% of this surgical series. Despite metastatic disease that limits longevity, operative survivors had improvement in functional capacity. Cardiac surgery should be considered for select patients with carcinoid heart disease affecting left- and right-sided valves.

Early and late results of operations for defects associated with corrected transposition and other anomalies with atrioventricular discordance in a pediatric population.

OBJECTIVES: The purpose of this study was to determine the early and late results for children having operations for defects associated with corrected transposition of the great arteries and other anomalies with atrioventricular discordance. METHODS: Data on 111 children operated on from July 1, 1971, through January 31, 1996, including clinic records, operative reports, and follow-up visits and questionnaires, were analyzed with particular reference to variables associated with early and late mortality, reoperations, ventricular function, and status of the atrioventricular valves. RESULTS: Complex associated anomalies were common and included double-outlet right ventricle (n = 43) and situs abnormalities (n = 38). Overall early mortality was 16%; for the 29 patients operated on after 1986, early mortality was 3%. Early survival was adversely affected by patch repair of ventricular septal defect and early operative interval. Follow-up of the 93 early survivors extended to 26.5 years (mean 11.4 years). Overall survival was 77% (+/-4%) at 5 years and 67% (+/-5%) at 10 years. Late survival was adversely affected by prior operations, more severe preoperative functional class, and cardiac rhythm other than sinus. Reoperation was required for 38 (41%) patients, most commonly for conduit replacement (n = 22) or repair/replacement of the systemic ventricle atrioventricular valve (n = 13). CONCLUSIONS: These results can serve as a basis for comparison with newer surgical alternatives proposed for corrected transposition of the great arteries.

Doppler hemodynamics of 51 clinically and echocardiographically normal pulmonary valve prostheses.

OBJECTIVE: To determine the normal Doppler hemodynamics of various pulmonary valve prostheses (PVPs). PATIENTS AND METHODS: We retrospectively analyzed comprehensive Doppler echocardiographic examinations of 51 patients (mean age, 27.8 years; range, 1-59 years) with PVPs that were normal on clinical and 2-dimensional echocardiographic examinations to establish the normal hemodynamics of various types and sizes of PVPs. The earliest complete postoperative transthoracic echocardiogram was identified for each patient. Doppler examinations were analyzed for peak instantaneous velocity, right ventricular outflow tract velocity, and peak and mean systolic gradient. The frequency of prosthetic regurgitation was also noted. RESULTS: The average +/- SD peak instantaneous velocity for all PVPs was 2.24+/-0.6 m/s, with an average peak systolic gradient of 20.4+/-10.4 mm Hg and an average mean systolic gradient of 11.0+/-5.1 mm Hg. The mean right ventricular outflow tract velocity was 1.0+/-0.2 m/s. Pulmonary homografts were found to have significantly lower peak velocities (average, 1.8+/-0.6 m/s) than all heterografts combined (average, 2.4+/-0.5 m/s; P=.002). Prosthetic regurgitation was more common in pulmonary homografts (88%) than in heterografts combined (29%; P<.001). CONCLUSION: This study establishes the normal range for Doppler hemodynamics of various PVPs, specifically homografts and heterografts, in both pediatric and adult patients.

Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.

OBJECTIVES: The purpose of this study was to determine long-term outcome in adults with congenitally corrected transposition of the great arteries (CCTGA), with particular emphasis on systemic ventricular dysfunction and congestive heart failure (CHF). BACKGROUND: Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and CHF being relatively common in older adults. METHODS: Retrospective analysis of records of 182 patients from 19 institutions were reviewed to determine current status and possible risk factors for systemic ventricular dysfunction and CHF. Factors considered included age, gender, associated cardiac defects, operative history, heart block, arrhythmias and tricuspid (i.e., systemic atrioventricular) regurgitation (TR). RESULTS: Both CHF and systemic ventricular dysfunction were common in groups with or without associated cardiac lesions. By age 45, 67% of patients with associated lesions had CHF, and 25% of patients without associated lesions had this complication. The rates of systemic ventricular dysfunction and CHF were higher with increasing age, the presence of significant associated cardiac lesions, history of arrhythmia, pacemaker implantation, prior surgery of any type, and particularly with tricuspid valvuloplasty or replacement. Aortic regurgitation (a previously unreported problem) was also relatively common in this patient population. CONCLUSIONS: Patients with CCTGA are increasingly subject to CHF with advancing age; this complication is extremely common by the fourth and fifth decades. Tricuspid (systemic atrioventricular) valvular regurgitation is strongly associated with RV (anatomical right ventricle connected to aorta in CCTGA patients; systemic ventricle in CCTGA) dysfunction and CHF; whether it is causative or a secondary complication remains speculative.

Churg-Strauss syndrome complicated by eosinophilic endomyocarditis.

A 34-year-old woman with asthma had increasing dyspnea on exertion for 9 months and new-onset mononeuritis multiplex. An examination demonstrated sinus tachycardia, elevated jugular venous pressure, and a tender nonpulsatile liver. The leukocyte count was 15.8 x 10(9)/L, with 23% eosinophils. Echocardiography revealed a laminated thrombus obliterating much of the right ventricular cavity, with encasement of the tricuspid valve. Ultrafast computed tomography showed no evidence of pulmonary emboli. Biopsy specimens of skin nodules revealed extravascular palisading granulomas. The thrombus was refractory to corticosteroids, and right ventricular thrombectomy was performed. To our knowledge, this is the third reported case of Churg-Strauss syndrome with thrombotic complications from coexistent eosinophilic endomyocarditis. In an asthmatic patient with chronic dyspnea, eosinophilic tissue infiltration, and neuropathy, Churg-Strauss syndrome should be considered; evaluation for cardiac involvement may be warranted.

Diet drug-related cardiac valve disease: the Mayo Clinic echocardiographic laboratory experience.

OBJECTIVE: To describe the prevalence of diet drug-related valvular disease among our referral population and the association of valvular disease with duration of exposure to fenfluramine and phentermine in combination and to dexfenfluramine alone. PATIENTS AND METHODS: In this retrospective review of clinical and echocardiographic data, charts of patients referred for treatment of toxic effects of diet drugs were reviewed, and telephone interviews were conducted. RESULTS: Between June and December 1997, 191 patients (164 women, 27 men; mean age, 47 years) were referred for possible diet drug-related valvular disease. Twenty-eight (28%) of the 99 asymptomatic patients and 40 (43%) of the 92 symptomatic patients had abnormal echocardiographic findings. Valvular lesions among the 68 patients with abnormal echocardiographic findings included mild (or greater) aortic regurgitation in 55 patients (81%), moderate (or greater) mitral regurgitation in 12 (18%), and moderate (or greater) tricuspid regurgitation in 7 (10%). The Food and Drug Administration case definition of diet drug-related valvulopathy was noted in 31 % of this referral population. Of patients with valvulopathy, mean duration of therapy with fenfluramine and phentermine in combination and dexfenfluramine alone was 9 months and 5 months, respectively. Duration of therapy was not associated with presence or absence of disease. Five patients had surgical intervention for severe valvulopathy: 3 had mitral valve repair, 1 had mitral valve replacement, and 1 had aortic valve replacement. Pulmonary hypertension (>40 mm Hg) was found in 24 patients (13%), and 17 (71 %) had pulmonary hypertension in association with valvulopathy. CONCLUSION: This study demonstrated a 31% (60/191) prevalence of valvulopathy in patients with a history of diet drug exposure who were referred for echocardiographic evaluation. The most common finding was mild aortic regurgitation. Twenty-eight percent of asymptomatic patients had abnormal echocardiographic findings. This study emphasizes the spectrum of diet drug-related cardiac disease and the potential for valvulopathy in asymptomatic patients.

Severe aortic stenosis with low transvalvular gradient and severe left ventricular dysfunction:result of aortic valve replacement in 52 patients.

BACKGROUND: The outcome of aortic valve replacement in patients with severe aortic stenosis, low transvalvular gradient, and severe left ventricular dysfunction is not well known. METHODS AND RESULTS: Between 1985 and 1995, 52 patients with left ventricular ejection fraction (EF) < or =35% and aortic stenosis with transvalvular mean gradient <30 mm Hg underwent aortic valve replacement. The mean (+/-SD) preoperative characteristics included EF, 26+/-8%; aortic valve mean gradient, 23+/-4 mm Hg; aortic valve area, 0.7+/-0.2 cm(2); and cardiac output, 3.7+/-1.2 L/min. Simultaneous coronary artery bypass graft surgery was performed in 32 patients (62%). Perioperative (30-day) mortality was 21% (11 of 52 patients). Ten additional patients died during follow-up. Advanced age (P=0.048) and small aortic prosthesis size (P=0.03) were significant predictors of hospital mortality by univariate analysis. By multivariate analysis, the only predictor of surgical mortality was smaller prosthesis size. The only predictor of postoperative survival was improvement in postoperative functional class (P=0.04). Postoperative functional improvement occurred in most patients. Postoperative EF was assessed in 93% of survivors; 74% demonstrated improvement. Positive change in EF was related to smaller preoperative aortic valve area and female sex. CONCLUSIONS: Despite severe left ventricular dysfunction, low transvalvular mean gradient, and increased operative mortality, aortic valve replacement was associated with improved functional status. Postoperative survival was related to younger patient age and larger aortic prosthesis size, and medium-term survival was related to improved postoperative functional class.

Echocardiographic improvement over time after cessation of use of fenfluramine and phentermine.

OBJECTIVE: To determine the echocardiographic changes over time of valvular heart lesions in patients who took the weight loss drugs fenfluramine and phentermine. SUBJECTS AND METHODS: This prospective cohort study began at the termination of a randomized, double-blind, placebo-controlled weight loss trial of 18 obese women and 13 obese men (mean age, 42 years; mean body mass index, 33.4 kg/m2) who had been assigned randomly to treatment with fenfluramine and phentermine or to placebo. Echocardiograms were obtained at termination of the trial when fenfluramine was withdrawn from the market and 6 months later. They were interpreted independently by 3 cardiologists blinded to treatment assignment and temporal sequence of the echocardiograms. The main outcome measure was the change in drug-related valvular disease over time. RESULTS: One subject assigned to receive the drugs was lost to follow-up, and 3 subjects who did not meet a weight loss goal of 10 kg crossed over from placebo to drug treatment. Echocardiograms were obtained in 19 subjects who received the drugs and 11 subjects who received placebo, and 6-month follow-up echocardiograms were obtained in 15 subjects who received the drugs and 3 who received placebo. Subjects had taken fenfluramine and phentermine a mean of 41 weeks (range, 8-73 weeks). Five of 19 subjects who received the drugs (26%; 95% confidence interval, 7%-46%) and 1 of 11 who received placebo (9%) (odds ratio, 3.6; 95% confidence interval, 0.4-35.6) had findings that met criteria established for drug-related valvular disease. All 5 subjects (4 women and 1 man) receiving the drugs had mild aortic regurgitation, and 1 also had pulmonary hypertension (estimated pulmonary artery pressure, 59 mm Hg). Six months later, the echocardiographic findings had improved in all 5 subjects (P=.06), and 3 no longer met the criteria for drug-related valvular disease. Pulmonary artery pressures decreased to near normal in the subject with pulmonary hypertension (37 mm Hg). Overall, the echocardiographic valvular features improved in 8 of 15 subjects who received the drugs and had echocardiograms performed at both time periods (P=.008). CONCLUSIONS: Valvular heart disease did not appear to progress after cessation of use of fenfluramine and phentermine, and echocardiographic valvular features appeared to improve over time.

Obesity drugs and the heart.

Appetite suppressant-related pulmonary hypertension and valvular heart disease are established disorders. Currently, the mechanism of these disorders is not certain. An estimated 6 million Americans and 70 million persons worldwide have been exposed to fenfluramine and dexfenfluramine. The clinical significance and long-term prognosis of cardiovascular effects and, thus, the potential public health effect of these disorders are not known. Longitudinal studies are required to further evaluate these disease processes. In addition, although isolated cases of regression of pulmonary hypertension and valve disease have been reported after the cessation of appetite suppressant therapy, the natural history remains uncertain.

Noncompaction of the ventricular myocardium.

Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular recesses communicating with the left ventricular cavity. The disease uniformly affects the left ventricle, with or without concomitant right ventricular involvement, and results in systolic and diastolic ventricular dysfunction and clinical heart failure. Noncompaction was initially described in children. However, recent studies have characterized this disease in the adult population, in whom this process may be more prevalent than currently appreciated. We describe an illustrative case of isolated noncompaction of the ventricular myocardium in a 57-year-old woman with the typical clinical and echocardiographic features of the disease. The literature on the topic is reviewed.

Pregnancy among women with congenitally corrected transposition of great arteries.

OBJECTIVES: The outcome of pregnancy in congenitally corrected transposition of the great vessels was studied in 22 women. BACKGROUND: Women with congenitally corrected transposition of the great vessels often reach childbearing age. Although reports on the outcome of pregnancy in these women are available, the number of patients is small. METHODS: The medical and surgical databases at the Mayo Clinic were reviewed, and 36 women >16 years old with congenitally corrected transposition of the great vessels were identified. All of them were contacted, and 22 who had pregnancies were identified and the outcome of pregnancy was evaluated. RESULTS: Twenty-two women had 60 pregnancies resulting in 50 live births (83%). Forty-four deliveries (88%) were vaginal and 6 (12%) were by cesarean section. One delivery was premature at 29 weeks. There was one successful twin pregnancy. There were 11 unsuccessful pregnancies. One patient developed congestive heart failure late in pregnancy because of systemic atrioventricular valve regurgitation and required valve replacement in the early postpartum period. One patient had a total of 12 pregnancies, including 1 twin pregnancy and 2 unsuccessful pregnancies. She had multiple pregnancy-related complications, including toxemia, congestive heart failure, endocarditis and myocardial infarction (single coronary artery). No other serious pregnancy-related maternal complications and no pregnancy-related deaths occurred. The mean birth weight of the infants (n = 32) was 3.2 +/- 0.4 kg. None of the 50 live offspring have been diagnosed with congenital heart disease. CONCLUSIONS: Successful pregnancy can be achieved in most women with congenitally corrected transposition of the great arteries. The rate of fetal loss and maternal cardiovascular morbidity is increased. Because of the small number of births, the risk of congenital heart disease in offspring of women with congenitally corrected transposition of the great arteries is uncertain.

Carcinoid heart disease and carcinoid syndrome: successful surgical treatment.

Tumor debulking can greatly improve quality of life for patients with malignant carcinoid syndrome, but hepatic cytoreduction is confounded by carcinoid heart disease, which can cause postsinusoidal portal hypertension, thereby increasing the risk of death from hemorrhage during hepatic resection. We describe a patient with metastatic carcinoid syndrome and carcinoid heart disease who had repair of his carcinoid heart disease and, after improvement of right-sided heart function, had successful hepatic debulking of carcinoid metastases.