Biomedical subjects
Publications and source records attributed to Heidi M Connolly.
Because the venous drainage of the ovary bypasses the portal circulation, carcinoid heart disease in patients with primary ovarian carcinoid tumors may develop in the absence of liver metastasis. We describe 4 patients who presented with symptomatic carcinoid heart disease in association with primary ovarian carcinoid tumor.
Exercise intolerance in Ebstein's anomaly is usually attributed to desaturation secondary to right-to-left shunting as a result of a small or distorted left ventricle (LV), significant tricuspid valve regurgitation, right ventricular dysfunction, or a combination of these. We observed one boy (age 15 years) and two women (ages 20 and 29 years) with severe Ebstein's anomaly and strikingly abnormal LV myocardium resembling the features described for LV noncompaction. LV size and systolic function were normal in the two women; the boy had a dilated LV with severely diminished ejection fraction. The LV myocardium was found to be unusually coarse and hypertrabeculated, with small intertrabecular recesses and an irregular endocardial surface. The findings in these 3 patients represent the whole spectrum of mild to severe LV noncompaction. Diastolic dysfunction was present in 2 of the 3 patients. Exercise tolerance was diminished in all. There was no mitral or aortic valve disease. The 15-year-old boy underwent heart transplantation 6 months later for biventricular failure. Thus, Ebstein's anomaly does not seem to be a pathology confined to the right ventricle, but may rarely lead to LV noncompacted myocardium. This LV pathology may be an additional explanation for exercise intolerance or signs of left heart failure in patients with Ebstein's anomaly.
OBJECTIVE: To review our experience with, and profile the safety and efficacy of, the Amplatzer PFO (patent foramen ovale) occluder (APO) and Amplatzer septal occluder (ASO) used to close PFO and/or atrial septal defect (ASD) in patients with paradoxical embolism (PE). PATIENTS AND METHODS: Between April 1998 and November 2002, 103 patients at the Mayo Clinic in Rochester, Minn, and Scottsdale, Ariz, mean age 52.4 years, with presumed PE (transient ischemic attack [n=22], stroke [n=77], or peripheral emboli [n=4]) underwent transcatheter device closure of PFO (n=81), ASD (n=12), and ASD/PFO (n=10) with 106 devices (APO [n=22] or ASO [n=84]). RESULTS: All devices deployed successfully, and no patients died. Procedural complications included atrial fibrillation (n=2), vessel injury (n=3), profound sinus node dysfunction (n=1), and device embolization with successful retrieval (n=1). At 3 months, 7 of 95 monitored patients had trivial residual shunt; at 12 months, 2 of 28 monitored patients had trivial residual shunt. Three patients had recurrent events--2 transient ischemic attacks and 1 retinal artery occlusion--at a mean +/- SD follow-up of 8.3 +/- 8.1 months (range, 1-34 months). None of these 3 patients had residual shunt or evidence of intracardiac thrombus. The average annual recurrence of all events was 3.6% at 23 months. The overall mean +/- SD freedom from recurrence of all events was 98.9% +/- 1.2% and 83.8% +/- 10.2% at 12 and 29 months of follow-up, respectively. CONCLUSIONS: Transcatheter device closure of PFO and/or ASD with use of APO/ASO in patients with presumed PE is effective and safe. Recurrent events may occur in the absence of a residual shunt.
Explore the source record for details and available documents.
BACKGROUND: By releasing vasoactive substances into the circulation, carcinoid tumors can cause right-sided valvular heart disease. Factors associated with the progression of carcinoid heart disease are poorly understood. We conducted a retrospective study to identify such factors. METHODS: Our sample included 71 patients with the carcinoid syndrome who underwent serial echocardiographic studies performed more than one year apart and 32 patients referred directly for surgical intervention after an initial echocardiographic evaluation. A score for carcinoid heart disease was determined on the basis of an assessment of valvular anatomy and function and the function of the right ventricle. An increase of more than 25 percent in the score between studies was considered suggestive of disease progression. Tumor progression was assessed on the basis of abdominal computed tomographic scans and changes in the level of urinary 5-hydroxyindoleacetic acid (5-HIAA), a metabolite of serotonin. RESULTS: Of the patients with serial echocardiographic studies, 25 (35 percent) had an increase of more than 25 percent in the cardiac score. As compared with patients whose score changed by 25 percent or less, these patients had higher urinary peak 5-HIAA levels (median, 265 mg per 24 hours [interquartile range, 209 to 593] vs. 189 mg per 24 hours [interquartile range, 75 to 286]; P=0.004) and were more likely to have biochemical progression (10 of 25 patients vs. 9 of 46, P=0.05) and to have received chemotherapy (13 of 25 vs. 10 of 46, P=0.009). Logistic-regression analysis showed that a higher peak urinary 5-HIAA level and previous chemotherapy were predictors of an increase in the cardiac score that exceeded 25 percent (odds ratio for each increase in 5-HIAA of 25 mg per 24 hours, 1.08 [95 percent confidence interval, 1.03 to 1.13]; P=0.009); odds ratio associated with chemotherapy, 3.65 [95 percent confidence interval, 1.74 to 7.48]; P=0.001). CONCLUSIONS: Serotonin is related to the progression of carcinoid heart disease, and the risk of progressive heart disease is higher in patients who receive chemotherapy than in those who do not.
OBJECTIVE: To determine the frequency of intracranial aneurysms (IAs) detected in patients with coarctation of the aorta (CoA) with use of magnetic resonance angiography. PATIENTS AND METHODS: From January 1, 1980, to September 30, 2002, 277 adult patients with CoA were seen at the Mayo Clinic in Rochester, Minn, and were invited to participate in a study to detect IAs. Of these 277 patients (mean +/- SD age, 41.6 +/- 16.5; 70 men), 100 underwent cranial magnetic resonance angiography. RESULTS: Ten patients had an IA (95% confidence interval, 5%-18%), with a mean diameter of 3.5 mm (range, 2.0-8.0 mm). The frequency of IA was significantly higher than that predicted in the general population (10% vs 2%; P < .001). One patient had surgical intervention. No clinical or geographical differences were found between patients with CoA and IA and patients with CoA and no IA. CONCLUSIONS: The frequency of IA among patients with CoA is approximately 5-fold that of the general population. Although no risk factors were identified in this cohort, additional prospective evaluation is warranted. These data suggest that noninvasive cerebral imaging to screen for IA should be considered in patients with CoA.
OBJECTIVE: We sought to investigate the clinical and echocardiographic (echo) characteristics of metastatic carcinoid tumor in the heart. BACKGROUND: Right-sided valvular dysfunction is the hallmark of carcinoid heart disease. Cardiac metastases are uncommon in carcinoid syndrome. Features of patients with metastatic carcinoid tumor involving the heart (MCH) have not been well described. METHODS: From 1985 through 1999, 11 patients (8 male, 3 female), mean age +/- standard deviation, 58 +/- 6 years, were seen who had pathologically confirmed MCH. All patients had echoes, which were reviewed retrospectively. RESULTS: All patients with MCH had carcinoid syndrome. The primary carcinoid tumor was in the small bowel in 83% of patients, and all patients had hepatic metastases. On pathologic review, the 11 patients had 15 MCH tumors. All metastases were intramyocardial. The MCH involved the right ventricle in 40%, left ventricle in 53%, and ventricular septum in 7%. The average size of macroscopic tumors was 1.8 +/- 1.2 cm. Nine MCH tumors were detected by echo in 6 of the 11 patients (55%). Mean echo-detected tumor size was 2.4 cm (range, 1.2 to 4). All tumors noted by echo were well circumscribed, non-infiltrating, and homogeneous. In the 5 other patients, review of autopsy records revealed 6 macroscopic tumors, mean size 0.35 cm (range, 0.2 to 0.4), none detected by echo even retrospectively. Carcinoid valve disease was present in 8 of the 11 MCH patients. The tricuspid valve was affected in all 8 patients (73%), pulmonary valve in 7 (64%), and left sided valves in 4 (36%) All patients with MCH identified by echo had cardiac surgery, 3 primarily for carcinoid valve disease and 2 for non-carcinoid cardiac disease; in 1 patient, MCH was the primary indication for cardiac surgery. CONCLUSIONS: MCH is uncommon but can be easily identified by echo if tumor size is >/=1.0 cm. In patients without valvular dysfunction, MCH may be the only manifestation of carcinoid heart disease. A search for MCH should be an integral part of the echo exam in patients with carcinoid syndrome.
BACKGROUND: Carcinoid heart disease characteristically affects tricuspid (TV) and pulmonary valves (PV), and TV replacement is helpful in selected patients. There is uncertainty, however, regarding optimal surgical management of PV regurgitation. METHODS AND RESULTS: We reviewed 22 patients having operation for carcinoid heart disease and compared those having TV and PV replacement (n=12), to those who underwent TV replacement and excision of the PV (n=10). Pre- and postoperative right ventricular (RV) size and dysfunction were assessed by consensus of 2 echocardiographers blinded to type of surgical treatment. RV dysfunction was graded as none (0), mild (1), moderate (2), or severe (3). RV size was graded as normal (0), or mild (1), moderate (2), or severe (3) enlargement. Preoperatively, RV size (2.2+/-0.8 [no PVR]versus 2.7+/-0.6 [with PVR], P=0.15), RV dysfunction (0.9+/-0.9 [no PVR]versus 1.4+/-0.7 [with PVR], P=0.14), and NYHA class were similar in the 2 groups. Postop RV size decreased inpatients with PVR, 2.7+/-0.6 to 1.7+/-1.0 (P=0.008), but did not change appreciably in those without PVR, 2.2+/-0.8 to 2.3+/-0.8 (P=0.67). There was no significant change in RV dysfunction after surgery, 1.4+/-0.7 to 1.8+/-0.9 with PVR (P=0.26) and 0.9+/-0.9 to 1.6+/-0.9 without PVR (P=0.07). CONCLUSIONS: PV replacement appears to have a beneficial effect on RV size in patients after surgery for carcinoid heart disease. This may have important implications for RV remodeling after PV replacement.
BACKGROUND: Although aortic valve replacement can be performed at an acceptable risk level in selected patients with left ventricular systolic dysfunction and low-output, low-gradient aortic stenosis, not all patients presenting with these hemodynamics will benefit from the operation. Some patients may have only mild aortic stenosis, despite a small calculated valve area. We report on the clinical utility of diagnostic dobutamine stimulation during cardiac catheterization in these diagnostically challenging patients. METHODS AND RESULTS: Thirty-two patients with low-output, low-gradient aortic stenosis and an ejection fraction <40% had dobutamine infusion in the catheterization laboratory. On the basis of the results of the dobutamine test, 21 patients underwent aortic valve replacement. All patients with a final aortic valve area < or =1.2 cm2 at peak dobutamine infusion and a mean gradient of >30 mm Hg were found to have severe calcific aortic stenosis at operation. In the 15 patients in whom contractile reserve was identified during dobutamine challenge (increase in stroke volume >20%), 1 patient died perioperatively (7% mortality) and 12 patients were alive in New York Heart Association class I or II status at follow-up. CONCLUSIONS: In patients with left ventricular systolic dysfunction and aortic stenosis with a low output and a low mean gradient, dobutamine challenge may aid in selecting those who would benefit from an aortic valve operation.
OBJECTIVES: The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries. BACKGROUND: The presentation of this disorder and the outcome in unoperated adults have not been well defined. METHODS: All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome. RESULTS: Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001). CONCLUSIONS: Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.
Literature has been limited in regard to the mechanisms of tricuspid regurgitation (TR) in patients with paramembranous (perimembranous) ventricular septal defect (VSD). Most observations have noted tricuspid valve clefts or dysplasia. We describe another mechanism for production of TR in association with paramembranous VSD. In 8 patients, we found significant TR produced by the VSD jet pushing the tricuspid anterior leaflet forward to open the tricuspid valve orifice. In these patients, a moderate paramembranous VSD extended slightly below the septal tricuspid leaflet with only partial obstruction of the VSD jet. All patients had restrictive VSD with low right ventricular pressure. This mechanism to produce TR was best defined by intraoperative transesophageal echocardiography, but current higher resolution imaging should allow correct diagnosis. We believe that when this mechanism for TR is found in association with a moderate VSD, surgical VSD closure is warranted.
OBJECTIVE: This study was undertaken to further define the impact of intraoperative transesophageal echocardiography during surgery for congenital heart disease and to determine appropriate indications. METHODS: The impact of transesophageal echocardiography on patient care was assessed in 1002 patients who underwent this procedure during surgery for congenital heart defects. It had major impact when new information altered the planned procedure or led to a revision of the initial repair. The safety of intraoperative transesophageal echocardiography was evaluated by review of the prospective data sheets and the medical record. A simple relative cost analysis was also performed. RESULTS: Patient median age was 9.9 years (range 2 days to 85 years). Transesophageal echocardiography had prebypass or postbypass major impact in 13.8% of cases (n = 138/1002). Major impact was more frequent during reoperations (P <.03). Procedures that benefited most from the additional information were valve repairs (aortic or atrioventricular) and complex outflow tract reconstructions. Partial anomalous pulmonary venous connection, tricuspid valve repair (other than of Ebstein anomaly), simple atrioventricular discordance, aortic arch anomalies, and secundum atrial septal defects had major impact rates less than 5%. No major complications occurred. Minor complications occurred in 1% of patients and were most often observed in infants smaller than 4 kg. Routine use of transesophageal echocardiography for all patients with congenital heart defects proved cost-effective. CONCLUSIONS: On the combined basis of the observed rates of major impact, the minimal complications, and the relative cost advantage, we believe that routine use of transesophageal echocardiography during most intracardiac repairs of congenital heart defects is justified, particularly for patients undergoing repeat operations for congenital cardiac malformations.
OBJECTIVE: To determine whether an association exists between pergolide and valvular heart disease. PATIENTS AND METHODS: Three patients with severe, unexplained tricuspid regurgitation were examined at our institution from September 2000 to April 2002. Echocardiography and histology of surgically explanted valves revealed abnormalities suggestive of carcinoid involvement, methysergide or ergotamine treatment, or use of fenfluramine and dexfenfluramine. Carcinoid valvular heart disease was excluded. None of the patients had prior treatment with these drugs. All 3 patients were taking pergolide. RESULTS: Of the 3 patients, 2 had predominantly right-sided congestive heart failure. In all 3 patients, echocardiography showed unusual valve morphology, with severe tricuspid valve regurgitation. Significant left-sided valve regurgitation was noted in 2 patients. Histologic analysis revealed surface fibroproliferative lesions with preserved underlying valve architecture. CONCLUSION: The echocardiographic and histopathologic features of these cases are strikingly similar to those associated with carcinoid-, ergot-, and fenfluramine-induced valve disease. An association between pergolide and valvular heart disease may therefore exist.
OBJECTIVE: To quantitate the pathologic features of carcinoid plaques in a relatively large number of surgical specimens from a single institution. PATIENTS AND METHODS: Medical records, operative reports, and surgical specimens were reviewed from all patients with carcinoid heart disease who underwent cardiac valvular surgery at Mayo Clinic, Rochester, Minn, between 1980 and 2000. RESULTS: The study group included 75 patients (45 men, 30 women) who ranged in age from 26 to 78 years (mean, 59 years). From these 75 patients, 139 valves had been excised surgically (73 tricuspid, 55 pulmonary, 6 mitral, 5 aortic). Pure regurgitation was the most common dysfunctional state of the tricuspid valve (80% [60/75]), mitral valve (97% [32/33]), and aortic valve (96% [23/24]). The pulmonary valve was more often both stenotic and insufficient (52% [37/71]) than purely regurgitant (30% [21/71]). In all cases, valve dysfunction was attributed to the presence of carcinoid plaques, which caused both thickening and retraction. Thickening was the result of both cellular proliferation and deposition of extracellular matrix. Proliferation of myofibroblasts was observed in all plaques and was mild in 49% (68/139) and moderate or severe in 51% (71/139). Extracellular matrix included collagen (in 99% of the 139 valves), myxoid ground substance (98% [136/139]), and elastin (20% [28/139]). Carcinoid plaques were also involved by neovascularization (94% [131/139]), chronic inflammation (94% [131/139]), and mast cell infiltration (64% [89/139]). Severe thickening was attributable primarily to collagen deposition in tricuspid valves and to myofibroblast proliferation and myxoid matrix in pulmonary valves. CONCLUSIONS: Among patients undergoing valvular surgery for carcinoid heart disease, tricuspid and pulmonary valves represented 92% of the excised valves (128/ 139). Although numerous cellular and extracellular features were common to the carcinoid plaques, variability in their relative expression produced appreciable differences in the histologic appearance among the plaques.
OBJECTIVE: To determine the indications for and spectrum of late reoperations in adults who had previously undergone coarctation repair. PATIENTS AND METHODS: We reviewed clinical, cardiac catheterization, and echocardiographic data and criteria for reoperation, surgical procedures, and outcome in 43 patients who underwent 54 reoperations between 1972 and 1996. RESULTS: Of the reoperations for recoarctation or associated cardiovascular disease (or both), 20% were performed in asymptomatic patients and 80% in symptomatic patients. Associated cardiovascular disease included bicuspid aortic valve in 36 patients (84%), aortic arch hypoplasia in 12 (28%), true or false aortic aneurysm in 6 (14%), mitral valve disease in 6 (14%), and subvalvular aortic stenosis in 5 (12%). Surgical procedures included 22 recoarctation repairs and 32 other cardiovascular interventions. Simultaneous repair of recoarctation and associated cardiovascular disease was performed as a single-stage repair in 5 reoperations through a median sternotomy using an extra-anatomic, ascending-to-descending aortic bypass, with no complications. One patient died (surgical mortality, 1.9%) of preexisting severe pulmonary vascular obstructive disease. CONCLUSIONS: After coarctation repair, associated cardiovascular diseases are the most common cause for reoperation. An individualized surgical approach is important and may range from valve replacement or recoarctation surgery to extra-anatomic bypass combined with other cardiovascular procedures, enabling simultaneous repair of recoarctation and associated lesions. Despite complex surgical techniques and multiple reoperations, morbidity and mortality were low in our series.