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Biomedical subjects

H M Connolly

Publications and source records attributed to H M Connolly.

34 records · Page 2Linked to original sources

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.

Diagnosis, Differential↗

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 Echocardiograms were obtained at termination of the trial when fenfluramine was withdrawn from the market and 6 months later.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.

Adrenergic Agents↗

Valvular heart disease associated with fenfluramine-phentermine.

BACKGROUND: Fenfluramine and phentermine have been individually approved as anorectic agents by the Food and Drug Administration (FDA). When used in combination the drugs may be just as effective as either drug alone, with the added advantages of the need for lower doses of each agent and perhaps fewer side effects. Although the combination has not been approved by the FDA, in 1996 the total number of prescriptions in the United States for fenfluramine and phentermine exceeded 18 million. METHODS: We identified valvular heart disease in 24 women treated with fenfluramine-phentermine who had no history of cardiac disease. The women presented with cardiovascular symptoms or a heart murmur. As increasing numbers of these patients with similar clinical features were identified, there appeared to be an association between these features and fenfluramine-phentermine therapy. RESULTS: Twenty-four women (mean [+/-SD] age, 44+/-8 years) were evaluated 12.3+/-7.1 months after the initiation of fenfluramine-phentermine therapy. Echocardiography demonstrated unusual valvular morphology and regurgitation in all patients. Both right-sided and left-sided heart valves were involved. Eight women also had newly documented pulmonary hypertension. To date, cardiac surgical intervention has been required in five patients. The heart valves had a glistening white appearance. Histopathological findings included plaque-like encasement of the leaflets and chordal structures with intact valve architecture. The histopathological features were identical to those seen in carcinoid or ergotamine-induced valve disease. CONCLUSIONS: These cases arouse concern that fenfluramine-phentermine therapy may be associated with valvular heart disease. Candidates for fenfluramine-phentermine therapy should be informed about serious potential adverse effects, including pulmonary hypertension and valvular heart disease.

Adult↗

Aortic valve replacement for aortic stenosis with severe left ventricular dysfunction. Prognostic indicators.

BACKGROUND: Aortic valve replacement for aortic stenosis (AS) carries an increased risk in the presence of left ventricular (LV) systolic dysfunction. Few data are available on the outcome of such patients. METHODS AND RESULTS: Between 1985 and 1992, 154 consecutive patients (107 men and 47 women) with LV systolic dysfunction (ejection fraction [EF] < or = 35%) underwent aortic valve replacement for AS. The mean preoperative characteristics included EF, 27 +/- 6%; aortic valve mean gradient, 44 +/- 18 mm Hg; aortic valve area, 0.6 +/- 0.2 cm2; and cardiac output, 4.1 +/- 1.5 L/min. Simultaneous coronary artery bypass graft surgery was performed in 78 patients (51%). Perioperative (30-day) mortality was 9% (14 of 154 patients). Fifty patients died during follow-up. Coronary artery disease (P = .002) and a reduced preoperative cardiac output (P = .03) were significantly related to reduced overall survival rate by multivariate analysis. Postoperative improvement occurred in most patients; 88% were New York Heart Association class III or IV before surgery versus 7% after surgery. Postoperative EF was assessed in 76% of survivors; 76% of these demonstrated improvement. By multivariate analysis, change in EF was inversely related to coronary disease (P = .002) and preoperative aortic valve area (P = .03). CONCLUSIONS: Despite LV dysfunction, the risk of aortic valve replacement for AS was acceptable and related to coronary artery disease and mean aortic gradient, and long-term survival was related to coronary disease and cardiac output. Improvement in symptoms and EF occurred in most patients.

Adult↗

Outcome of pregnancy in patients with complex pulmonic valve atresia.

Fifteen women with complex pulmonary atresia who had pregnancies were retrospectively reviewed. Although no pregnancy-related deaths occurred, complications were noted in 3 patients and risk of fetal loss and premature birth were increased, none of the 10 offspring had congenital heart disease.

Abortion, Spontaneous↗

Mimics of Ebstein's anomaly.

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.

Adolescent↗

Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography.

OBJECTIVE: This study sought to demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE). BACKGROUND: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly whose diagnosis has classically been made using angiography. METHODS: We performed a retrospective review of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume overload or suspected intracardiac shunting by transthoracic echocardiography or intraoperatively. RESULTS: A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation. CONCLUSIONS: TEE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence the need for medical and surgical management. TEE should be performed in patients with right ventricular volume overload when the precordial examination is inconclusive.

Adult↗

Transesophageal echocardiography: unusual case of anomalous pulmonary venous connection to the azygos vein.

Partial anomalous pulmonary venous connection, a rare congenital anomaly, most commonly involves the right lung, with one or more pulmonary veins anomalously connecting most frequently to the superior vena cava and less commonly to the right atrium or inferior vena cava. This article describes an unusual case of anomalous pulmonary venous connection of the right lung to the azygos vein in an adult. This anomaly was clearly delineated with angiography, computed tomography of the chest, and transesophageal echocardiography. The transesophageal echocardiographic features of the anomaly are described as a means to prevent further diagnostic misinterpretation.

Angiography↗

Coarctation of the aorta: collateral flow assessment with phase-contrast MR angiography.

OBJECTIVE: The purpose of this report is to describe a new use of MR imaging in coarctation of the aorta. The specific question addressed was how well collateral blood flow in intercostal arteries, as determined by phase-contrast MR angiography, correlated with findings during surgery or catheterization in patients with coarctation of the aorta. CONCLUSION: Phase-contrast MR angiography is an excellent technique for detecting the presence or absence of collateral blood flow in the intercostal arteries of patients with coarctation of the aorta. Knowing whether collateral blood flow is present in patients with narrowing of the juxtaductal aorta should help assess the clinical hemodynamic significance of the coarctation.

Adult↗

Outcome of cardiac surgery for carcinoid heart disease.

OBJECTIVES: The hypothesis was that cardiac surgery for symptomatic carcinoid heart disease in conjunction with adjunctive therapy could improve the long-term outlook of patients with carcinoid heart disease. BACKGROUND: Patients with carcinoid heart disease have a dismal prognosis; most die of progressive right heart failure within 1 year after onset of symptoms. Improved therapies for the systemic manifestations of the carcinoid syndrome have resulted in symptomatic improvement and prolonged survival in patients without heart disease. METHODS: Twenty-six patients with symptomatic carcinoid heart disease underwent valvular surgery. Preoperative clinical, laboratory, Doppler echocardiographic and hemodynamic factors were evaluated. The survival of the surgical group was compared with that of a control group of 40 medically treated patients. RESULTS: There were nine perioperative deaths (35%), primarily from postoperative bleeding and right ventricular failure. Of the 17 surgical survivors, 8 were alive at a mean of 28 months of follow-up. The postoperative functional class of the eight surviving patients was substantially improved. Late deaths were primarily due to hepatic dysfunction caused by metastatic disease. The only predictor of operative mortality (p = 0.03) was low voltage on preoperative electrocardiography (limb lead voltage < or = 5 mm). Predictors of late survival included a lower preoperative somatostatin requirement and a lower preoperative urinary 5-hydroxy-indoleacetic acid level. There was a trend toward increased survival for the surgical group compared with the control group. CONCLUSIONS: Because new therapies have improved survival in patients with the malignant carcinoid syndrome, cardiac involvement has become a major cause of morbidity and mortality. Valve surgery is the only definitive treatment. Although cardiac surgery carries a high perioperative mortality, marked symptomatic improvement occurs in survivors. Surgical intervention should therefore be considered when cardiac symptoms become severe.

Actuarial Analysis↗

Ebstein's anomaly: outcome of pregnancy.

OBJECTIVES: The outcome of pregnancy in Ebstein's anomaly was studied in 72 such patients (44 women, 28 men) who had had pregnancies or fathered children. BACKGROUND: Patients with Ebstein's anomaly often reach childbearing age. Reports of the outcome of pregnancy in Ebstein's anomaly are available; however, the number of patients is small. METHODS: The medical and surgical data bases at the Mayo Clinic were reviewed, and 145 patients (62 men, 83 women) of childbearing age with Ebstein's anomaly were located. All patients were contacted, and 72 patients (44 women, 28 men) with offspring were identified and reviewed in detail to assess the outcome of pregnancy. RESULTS: Forty-four women had 111 pregnancies resulting in 85 live births (76%). Seventy-six deliveries (89%) were vaginal, and nine (11%) were by cesarean section. Twenty-three deliveries were premature. There were 19 spontaneously unsuccessful pregnancies, 7 therapeutic abortions and 2 early neonatal deaths. The mean birth weight of the infants born to cyanotic women was 2.53 kg, which was significantly lower than the mean birth weight of infants born to acyanotic women (3.14 kg [p < 0.001]). The overall incidence of congenital heart disease in the 158 offspring of parents with Ebstein's anomaly was 4% (6 of 158). The incidence of congenital heart disease was 6% (5 of 83) in the offspring of women with Ebstein's anomaly and 1% (1 of 75) in that of men. There was a 0.6% (1 of 158) incidence of familial Ebstein's anomaly. There were no serious pregnancy-related maternal complications, which included maternal death, stroke, congestive heart failure, arrhythmias or endocarditis. CONCLUSIONS: Pregnancy in women with Ebstein's anomaly is well tolerated. It is associated with an increased risk of prematurity, fetal loss and congenital heart disease in the offspring. In addition, a significantly lower birth weight is found in the offspring of cyanotic versus acyanotic women with Ebstein's anomaly. Paternal Ebstein's anomaly also seems to result in an increased risk of congenital heart disease in the offspring compared with the incidence in the general population.

Adolescent↗

Doppler hemodynamic profiles of 82 clinically and echocardiographically normal tricuspid valve prostheses.

BACKGROUND: Normal Doppler hemodynamics for tricuspid prostheses have not been well characterized in a large group of patients. Therefore, we analyzed comprehensive Doppler echocardiographic examinations of 82 patients with tricuspid prostheses that were normal by clinical and two-dimensional echocardiographic examinations to establish the normal hemodynamics of various types and sizes of tricuspid prostheses. METHODS AND RESULTS: The earliest complete postoperative echocardiographic study from each patient was chosen for analysis. Doppler examinations were analyzed on an off-line station from tapes or Doppler strip charts. Early velocity, atrial velocity, end-diastolic velocity, pressure half-time, and mean gradient were obtained by digitizing tricuspid velocity curves. The incidence of "physiological" tricuspid prosthetic regurgitation was noted. Ten Doppler cycles were measured for each patient, and maximal, minimal, and average measurements were recorded. The mean values +/- SD of early velocity, atrial velocity, end-diastolic velocity, mean gradient, and pressure half-time and incidence of mild prosthetic regurgitation were reported for each type of prosthesis, as were highest Doppler measurements for each valve type. Average pressure half-time was significantly lower for St Jude than for heterograft prostheses (P = .04). There were no significant differences between the valve types for mean gradient, early velocity, or incidence of prosthetic regurgitation. Increasing prosthesis size was associated with lower average pressure half-time for heterograft prostheses (P = .024). Average differences (respiratory- and cycle-length-dependent) between maximal and minimal values for 10 cardiac cycles were established for each prosthesis. CONCLUSIONS: This study establishes normal ranges for Doppler hemodynamics of various tricuspid prostheses and emphasizes the importance of measuring multiple cycles for each tricuspid prosthesis, regardless of cardiac rhythm.

Adolescent↗

Blood flow to the rat rectum: its intramural distribution and the response to injury.

Rectal blood flow was measured in rats using radioactive microspheres. Subsequent sectioning of the material allowed the intramural distribution of that flow to be assessed. Total flow increased with increasing distance from the anorectal junction but the distribution of flow remained the same with the mucosa receiving slightly less than two thirds of the total. Injury to the mucosa, produced by a surfactant administered as a suppository, doubled total flow in areas where the damage was most severe and revealed an ability of the intramural vascular beds to react independently. Flow to the mucosa increased both absolutely and proportionately and this entirely accounted for the increase in total flow. Flow to the muscularis externa was unaltered in absolute terms, but fell as a percentage of total flow.

Adrenal Glands↗

Congenital absence of the pericardium: echocardiography as a diagnostic tool.

Between 1982 and 1992, 10 patients who underwent echocardiography at the Mayo Clinic were found to have congenital absence of the pericardium. Clinical, electrocardiographic, chest roentgenographic, echocardiographic, computed tomographic, and magnetic resonance imaging features were reviewed in this patient group. The characteristic features of this entity are reviewed. The echocardiographic features in order of frequency are (1) unusual echocardiographic windows, seen in all 10 patients, (2) cardiac hypermobility in nine patients, (3) abnormal ventricular septal motion in eight patients, and (4) abnormal swinging motion of the heart in seven patients.

Adolescent↗