Biomedical subjects
Heidi M Connolly
Publications and source records attributed to Heidi M Connolly.
Carcinoid heart disease in patients without hepatic metastases.
Most carcinoid tumors originate in the gut. Carcinoid heart disease typically occurs when tumor progression results in the formation of hepatic metastases, which allow vasoactive substances to reach the heart without being metabolized in the liver. Except for patients with primary ovarian carcinoid tumors, the occurrence of carcinoid heart disease without hepatic metastases has been reported only anecdotally. From a retrospective analysis of 265 patients, 4 patients were identified who developed carcinoid heart disease in the absence of liver metastases or primary tumors located in the ovaries. All 4 patients had metastases to the retroperitoneal lymph nodes and had carcinoid syndrome. The reasons for referral to cardiac evaluation by transthoracic echocardiography were findings on auscultation in 3 patients and exertional dyspnea in 1 patient. In conclusion, cardiac symptoms or findings on auscultation should prompt further evaluation by transthoracic echocardiography in these patients, although the classic prerequisites for development of carcinoid heart disease are lacking.
Gastrointestinal bleeding and protein-losing enteropathy after the fontan operation.
Gastrointestinal bleeding has not been reported in patients with protein-losing enteropathy. The investigators describe 3 patients with protein-losing enteropathy and gastrointestinal bleeding after Fontan operations. Bleeding occurred at the onset of protein-losing enteropathy, was macroscopic, led to anemia, and required blood transfusion in all patients. Invasive testing failed to demonstrate gastrointestinal pathology as a source of bleeding. In conclusion, unexplained gastrointestinal bleeding may be part of the clinical spectrum of protein-losing enteropathy.
Carcinoid heart disease.
Progress in the medical and surgical management of patients with carcinoid disease has resulted in improved symptoms and survival. Carcinoid heart disease remains a major cause of morbidity and mortality among patients with malignant carcinoid syndrome. Limited medical treatment options are available for patients with symptomatic carcinoid heart disease. At the Mayo Clinic (Rochester, MN), we have taken an aggressive approach to severe valvular dysfunction from carcinoid heart disease. Patients with severe carcinoid heart disease currently are referred for cardiac operation when they develop cardiac symptoms, ventricular dysfunction, or (rarely) in anticipation of hepatic surgery. Surgical outcome depends on patient age and functional class at the time of cardiac surgery. Despite metastatic disease that limits longevity, cardiac surgical survivors usually demonstrate dramatic improvement in functional capacity. Cardiac surgery should be considered early for patients with symptomatic carcinoid heart disease and controlled carcinoid symptoms. An experienced medical, surgical, and anesthetic team approach to the patient with carcinoid heart disease is critical in order to provide state of the art management.
Tako-tsubo cardiomyopathy in aneurysmal subarachnoid hemorrhage: an underappreciated ventricular dysfunction.
OBJECT: Neurogenic stunned myocardium in aneurysmal subarachnoid hemorrhage (SAH) is associated with a wide spectrum of reversible left ventricular wall motion abnormalities and includes a subset of patients with a pattern of apical akinesia and concomitant sparing of basal segments called "tako-tsubo cardiomyopathy". METHODS: After obtaining institutional review board approval, the authors retrospectively identified among all patients admitted to the Mayo Clinic's Neurological Intensive Care Unit between January 1990 and January 2005 those with aneurysmal SAH who had met the echocardiographic criteria for tako-tsubo cardiomyopathy. Among 24 patients with SAH-induced reversible cardiac dysfunction, the authors identified eight with SAH-induced tako-tsubo cardiomyopathy. All eight patients were women with a mean age of 55.5 years (range 38.6-71.1). Seven patients presented with a poor-grade SAH, reflected by a Hunt and Hess grade of III or IV. Four patients underwent aneurysm clip application, and four underwent endovascular coil occlusion. The initial mean ejection fraction (EF) was 38% (range 25-55%), and the mean EF at recovery was 55% (range 40-68%). Cerebral vasospasm developed in six patients, but cerebral infarction developed in only three patients. CONCLUSIONS: The authors describe the largest cohort with aneurysmal SAH-induced tako-tsubo cardiomyopathy. In the SAH population, tako-tsubo cardiomyopathy predominates in postmenopausal women and is often associated with pulmonary edema, prolonged intubation, and cerebral vasospasm. Additional studies are warranted to understand the complex mechanism involved in tako-tsubo cardiomyopathy and its intriguing relationship to neurogenic stunned myocardium.
Fate of bicuspid aortic valves in patients undergoing aortic root repair or replacement for aortic root enlargement.
OBJECTIVE: To better understand the outcome of the bicuspid aortic valve (BAV) after valve-sparing root replacement, we evaluated BAV function after its preservation at the time of the ascending aorta (AA) repair. PATIENTS AND METHODS: From January 1, 1994, through March 31, 2002, BAVs were preserved during repair of AA aneurysms in 21 patients (mean +/- SD age, 45+/-12 years; 13 males). We reviewed the clinical and surgical records of these patients for baseline characteristics, operative procedures, and data at latest follow-up. RESULTS: The primary indication for operation was AA enlargement (median diameter, 55 mm; range, 43-65 mm) in 18 patients and severe aortic regurgitation (AR) in 3 patients. Preoperatively, the BAV exhibited mild or moderate stenosis in 5 patients (mean gradient, 25 mm Hg; range, 19-34 mm Hg), moderate AR in 2, and severe AR in 3. Aortic valve repair was performed in 11 patients. Seventeen patients had excision and graft replacement of the enlarged AA, and 4 patients had primary AA repair. There were no perioperative deaths. Moderate or severe AR was relieved in all patients early postoperatively and during follow-up (median, 2.5 years; maximum, 7.6 years). One patient required aortic valve replacement at 4.8 years for AR. An additional patient had recurrent, severe AR but was asymptomatic and has not undergone reoperation. CONCLUSION: Repair of diseased BAVs or preservation of functionally normal BAVs during surgery for AA aneurysms has a low perioperative risk, and early results are encouraging. Additional observation is necessary to determine whether this strategy has real advantages over early aortic valve replacement.
Prognosis of carcinoid heart disease: analysis of 200 cases over two decades.
BACKGROUND: The long-term prognosis of patients who develop carcinoid heart disease and the effect of cardiac surgery on outcome are not well established. METHODS AND RESULTS: In this retrospective study, we identified 200 patients with carcinoid syndrome referred for echocardiography in whom the diagnosis of carcinoid heart disease was confirmed. Patients were divided into 3 groups of similar size according to the date from first diagnosis of carcinoid heart disease. Group A comprised patients diagnosed from 1981 through June 1989; group B, diagnosed July 1989 through May 1995; and group C, June 1995 through 2000. The end point was all-cause mortality. Median survival was significantly lower in group A (1.5 years, 95% CI 1.1 to 1.9 years) compared with groups B (3.2, 95% CI 1.3 to 5.1 years) and C (4.4, 95% CI 2.4 to 7.1 years; P=0.009). In a multivariate model adjusted for treatment and clinical characteristics, the risk of death in groups B (hazard ratio 0.67, 95% CI 0.46 to 0.99, P=0.04) and C (hazard ratio 0.61, 95% CI 0.39 to 0.92, P=0.006) was significantly reduced relative to group A. Cardiac surgery was performed in 87 patients. When cardiac surgery was included as a time-dependent covariate in a multivariate analysis, it was associated with a risk reduction of 0.48 (95% CI 0.31 to 0.73, P<0.001), whereas the time period of diagnosis was no longer significant. CONCLUSIONS: The prognosis of patients with recognized carcinoid heart disease has improved over the past 2 decades at our institution. This change in survival may be related to valve replacement surgery.
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
BACKGROUND: Sinus venosus atrial septal defect (SVASD) differs from secundum atrial septal defect by its atrial septal location and its association with anomalous pulmonary venous connection (APVC). Data on long-term outcome after surgical repair are limited. METHODS AND RESULTS: We reviewed outcomes of 115 patients (mean age+/-SD 34+/-23 years) with SVASD who had repair from 1972 through 1996. APVC was present in 112 patients (97%). Early mortality was 0.9%. Complete follow-up was obtained for 108 patients (95%) at 144+/-99 months. Symptomatic improvement was noted in 83 patients (77%), and deterioration was noted in 17 patients (16%). At follow-up, 7 (6%) of 108 patients had sinus node dysfunction, a permanent pacemaker, or both, and 15 (14%) of 108 patients had atrial fibrillation. Older age at repair was predictive of postoperative atrial fibrillation (P=0.033). No reoperations were required during follow-up. Survival was not different from expected for an age- and sex-matched population. Clinical improvement was more common with older age at surgery (P=0.014). Older age at repair (P=0.008) and preoperative New York Heart Association class III or IV (P=0.038) were independent predictors of late mortality. CONCLUSIONS: Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. Postoperative atrial fibrillation appears to be related to older age at operation. SVASD repair achieves survival similar to that of a matched population and should be considered whenever repair may impact survival or symptoms.
Severe symptomatic tricuspid valve regurgitation due to permanent pacemaker or implantable cardioverter-defibrillator leads.
OBJECTIVES: We report a series of patients with severe tricuspid valve regurgitation due to a permanent pacemaker (PPM) or implantable cardioverter-defibrillator (ICD) lead. BACKGROUND: Severe tricuspid regurgitation caused by a PPM or ICD lead is an under-recognized but treatable etiology of severe right heart failure. METHODS: We reviewed the records of 41 patients who underwent tricuspid valve operation for severe tricuspid regurgitation caused by previously placed PPM or ICD leads. RESULTS: During surgery, severe tricuspid regurgitation was found to be caused by the PPM or ICD leads in all 41 patients. There was a perforation of the tricuspid valve leaflet by the PPM or ICD lead in 7 patients, lead entanglement in the tricuspid valve occurred in 4 patients, lead impingement of the tricuspid valve leaflets occurred in 16 patients, and lead adherence to the tricuspid valve occurred in 14 patients. The septal leaflet was most often perforated (6 of 7). In the preoperative evaluation, valve malfunction due to the PPM or ICD lead was diagnosed preoperatively in only 5 of 41 (12%) patients by transthoracic echocardiography. All patients underwent successful tricuspid valve operation (22 tricuspid valve replacement), with one perioperative death occurring. During follow-up (range, 1 to 99 months), there was one patient who died from left-sided heart failure and three patients died of other causes. The remaining patients showed improvement in signs and symptoms of heart failure. CONCLUSIONS: Damage to the tricuspid valve by PPM or ICD leads may result in severe symptomatic tricuspid regurgitation and may not be overtly visualized by echocardiography. This etiology should be considered when evaluating patients with severe right heart failure after PPM or ICD implantation.
Prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies.
OBJECTIVES: This study was designed to determine the prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies and to assess the clinician's ability to predict the presence or absence of 22q11.2 microdeletion on the basis of clinical features. BACKGROUND: It is known that 22q11.2 microdeletion is a chromosomal anomaly with cardiac and extracardiac manifestations. The prevalence and manifestations in adults have not been well characterized. METHODS: A total of 103 consecutive adults with either tetralogy of Fallot (TOF), pulmonary atresia/ventricular septal defect (PA/VSD), or truncus arteriosus (TA) were prospectively screened for 22q11.2 microdeletion using a fluorescence in situ hybridization (FISH) assay. Clinicians were asked to predict 22q11.2 microdeletion status on the basis of clinical features. A geneticist blinded to FISH assay results reviewed photographs of the patients for typical dysmorphic features of 22q11.2 microdeletion. RESULTS: Six patients (prevalence 5.8%, 95% confidence interval 1.3 to 10.3) had 22q11.2 microdeletion (3 with TOF, 2 with PA/VSD, 1 with TA). In two of these patients, the clinician incorrectly predicted absence of the deletion. In three, typical dysmorphic features of 22q11.2 microdeletion were absent. CONCLUSIONS: Our work showed that 22q11.2 microdeletion is under-recognized in adults with congenital heart disease. The absence of typical phenotypic features makes it difficult to correctly predict if the deletion is present. Screening for 22q11.2 microdeletion should be considered in adults with high-risk cardiac lesions, as it has important implications in reproductive counseling and surveillance for associated extracardiac manifestations.
Pregnancy in women with congenital heart disease.
Congenital heart disease is the most common form of structural heart disease affecting women of childbearing age in developed countries. Pregnancy in these patients is associated with an increased risk to both mother and fetus. Appropriate prepregnancy evaluation and counseling is recommended to assess the pregnancy-related maternal and fetal risk and to identify patients who should avoid pregnancy. Once pregnancy occurs, cardiovascular reevaluation is generally recommended; the frequency is individualized. Monitoring during delivery may be necessary and the postpartum period is a concern in select individuals. Data regarding the outcome of pregnancy in patients with operated congenital cardiac defects are available. Individualized care is mandatory.
Doppler echocardiography of 119 normal-functioning St Jude Medical mitral valve prostheses: a comprehensive assessment including time-velocity integral ratio and prosthesis performance index.
The purpose of this study was to provide, in a large number of patients, comprehensive Doppler echocardiographic assessment of normal St Jude Medical mitral valve prosthesis function using Doppler-derived hemodynamic variables, including the mitral valve prosthesis-to-left ventricular outflow tract time-velocity integral ratio and prosthesis performance index. The pressure half-time was less than 130 milliseconds in all patients, and all but one patient had either a peak early mitral diastolic velocity of 2 m/s or less or a mitral valve prosthesis-to-left ventricular outflow tract time-velocity integral ratio of less than 2.2. There was a significant (P < .001) negative correlation between the prosthesis performance index and prosthesis size. This negative correlation suggests that there is more efficient use of the in vitro geometric orifice area with smaller prostheses.
Aortic root replacement with a novel decellularized cryopreserved aortic homograft: postoperative immunoreactivity and early results.
OBJECTIVE: Novel antigen-reduction SynerGraft technology (CryoLife Inc, Kennesaw, Ga) reduces the cellular components of a cadaver homograft with an enzymatic and washing process, leaving the extracellular matrix intact. We report the immunoreactivity (positive panel reactive antibody assay) and early operative results of the CryoLife SynerGraft aortic valve conduit homograft. METHODS: Twenty-two patients (age 53 +/- 14 years, range 31-80 years) from April 2002 to July 2003 underwent aortic root replacement with a CryoLife SynerGraft aortic valve conduit homograft (CryoLife Inc, Kennesaw, Ga) for congenital or acquired aortic valve disease, aortic aneurysm with aortic valve disease, or native or prosthetic aortic valve endocarditis. Baseline percentage positive panel reactive antibody results were negative (<10%) for all and were assessed at 1 month, 3 months, and 1-year. Homograft function was evaluated by echocardiography. RESULTS: Early mortality was 0%. Two late deaths at 1 postoperative year were unrelated to homograft function. At 1 postoperative month, panel reactive antibody results were negative in 20 patients (91%). At 3 months, 19 of 22 patients (86%) had negative results, including 1 with previous positive results. At 1 year, 19 of 20 patients (95%) had negative results, including 2 of the 3 with previous positive results. The mean aortic valve gradients were 12 +/- 8 mm Hg (n = 21) at discharge and 11 +/- 7 mm Hg (n = 18) at 1 year. At a mean follow-up of 30.3 +/- 5.2 months, the mean gradient was 8.8 +/- 6.3 mm Hg. CONCLUSION: The SynerGraft decellularization technology successfully removed antigens from an aortic valve homograft conduit. Aortic root reconstruction with the CryoLife SynerGraft aortic valve homograft resulted in low transvalvular gradients, similar to those seen with standard cryopreserved homografts. These early results suggest an advancement in homograft technology. The low panel reactive antibody response may enhance durability by eliminating immune complex-mediated reaction against the homograft. The lack of allosensitization in patients who may require organ transplantation in the future is an added benefit.
Acute renal failure after cardiac surgery for carcinoid heart disease: incidence, risk factors, and prognosis.
BACKGROUND: Cardiac surgery for carcinoid heart disease may be associated with acute renal failure (ARF) and multiorgan dysfunction postoperatively. This study was performed to determine the incidence, risk factors, and prognosis of ARF after cardiac surgery for carcinoid heart disease. METHODS: This is a case-control study of 86 consecutive patients who underwent cardiac surgery for carcinoid heart disease. ARF is defined as hemodialysis requirement or serum creatinine level 50% greater than baseline, resulting in an estimated creatinine clearance less than 40 mL/min (0.67 mL/s). Preoperative, operative, and postoperative characteristics were examined and compared between groups to determine risk factors and prognosis of ARF. RESULTS: ARF occurred in 22% of cases (19 of 86 patients). Preoperative characteristics and type of procedure did not differ between groups. Operative variables associated with ARF by means of univariate analysis included longer surgical and bypass times, intra-aortic balloon pump use, and perioperative epinephrine requirement. Postoperatively, ARF was associated with epinephrine use, prolonged mechanical ventilation, prolonged intensive care unit admission, and higher Acute Physiology and Chronic Health Evaluation II scores. Perioperative mortality in the ARF group was 47% versus 4.5% in the control group (odds ratio, 36.1; 95% confidence interval, 8.0 to 261.8). CONCLUSION: ARF developed in a relatively high proportion of this cohort, but traditional preoperative risks failed to predict post-cardiac surgery ARF. Variables closely related to perioperative hemodynamic compromise were associated with ARF. ARF portends a particularly poor prognosis in this cohort that is explained largely by multiorgan failure syndrome.
Left heart lesions in patients with Ebstein anomaly.
OBJECTIVE: To identify the incidence of left heart abnormalities in patients with Ebstein anomaly, recognizing that left-sided lesions in this patient group have been overlooked. PATIENTS AND METHODS: According to the echocardiography database at the Mayo Clinic in Rochester, Minn, 106 consecutive patients with Ebstein anomaly underwent echocardiography between July 1, 2001, and February 28, 2003. Clinical data as well as electrocardiographic and echocardiographic reports and images were reviewed. RESULTS: Ebstein anomaly was severe in 76 patients (72%). Previous tricuspid valve surgery was reported in 46 patients (43%), and previous closure of an atrial septal defect or patent foramen ovale was reported in 34 patients (32%). Left ventricular (LV) myocardial changes resembling noncompaction occurred in 19 patients (17.9%), LV systolic dysfunction in 7 patients (7%), LV diastolic dysfunction in 34 (36%) of 95 patients, and LV dilatation in 4 patients (4%). Additional left-sided cardiac lesions included mitral valve prolapse in 16 patients (15%), bicuspid aortic valve in 8 (8%), mitral valve dysplasia in 4 (4%), and ventricular septal defect in 8 (8%). Wolff-Parkinson-White syndrome occurred in 22 patients (21%). The QRS axis tended to be different in LV noncompaction with a mean +/- SD axis of 12 degrees +/- 74 degrees vs 36 degrees +/- 66 degrees overall (P=.08). Otherwise, there were no differences in clinical or surgical data between the groups with normal and abnormal LV myocardium. CONCLUSIONS: In patients with Ebstein anomaly, left heart abnormalities involving the myocardium or valves were observed in 39% of patients. Ebstein anomaly should not be regarded as a disease confined to the right side of the heart.
Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis.
OBJECTIVE: To determine the long-term outcome of patients after surgical repair for pulmonary valve stenosis (PVS). PATIENTS AND METHODS: Fifty-three patients (30 males; mean - SD age, 10+/-13 years; range, 5 days to 50 years) were identified who had surgical treatment for PVS between 1951 and 1982. The status of each patient was determined by medical record review. RESULTS: The mean +/- SD age at follow-up was 43+/-15 years (age range, 19-77 years). Mean follow-up was 33 years (range, 18-51 years). At a median follow-up of 34 years, 35 reinterventions had been performed in 28 patients (53%), Including pulmonary valve replacement for free pulmonary regurgitation in 21 patients (mean interval after initial surgery, 33 years; range, 14-45 years), open valvotomy in 5 and pulmonary balloon valvuloplasty in 3 for residual PVS, closure of atrial septal defect in 2, right ventricular outflow tract reconstruction in 1, closure of iatrogenic ventricular septal defect in 1, ligation of aortopulmonary fistula in 1, and tricuspid valve annuloplasty with simultaneous coronary artery bypass grafting in 1. In addition, atrial and ventricular arrhythmias were common, occurring in 20 patients (38%). Patients who underwent reintervention were more likely to have undergone closed pulmonary valvotomy as the initial repair (P=.008). CONCLUSION: Although overall survival after surgical treatment of isolated PVS remains excellent, many patients undergo late reintervention after 30 years of follow-up, emphasizing the need for lifelong cardiac follow-up.
Surgical management of the aortic root in patients with Marfan syndrome.
BACKGROUND AND AIM OF THE STUDY: Surgical aortic root reconstruction techniques are standard therapy to avoid catastrophic vascular events in patients with Marfan syndrome with a dilated and/or dissected aortic root. The study aim was to evaluate the long-term results of aortic root reconstruction. METHODS: Eighty-three patients (54 males, 29 females; mean age 37+/-17 years) fulfilling strict Ghent criteria for Marfan syndrome underwent aortic root surgery between 1971 and 2001. Of these patients, 65 (78%) underwent a composite valve conduit repair and 18 (22%) a valve-sparing aortic root reconstruction. Six patients (7%) suffered from an acute type A dissection, and 16 (19%) a chronic type A dissection. RESULTS: In-hospital and 30-day mortality was 3.6% (n = 3). Morbidity included stroke (1.2%; n = 1), perioperative myocardial infarction (1.2%; n = 1) and reoperation for bleeding (10%; n = 8). Of 21 late deaths, the cause was cardiac in nine cases. Actuarial survival at 5, 10, 15 and 20 years was 84% (95% CI 76-93%), 73% (CI 61-86%), 59% (CI 45-77%) and 43% (CI 26-72%), respectively. Multivariate predictors for late death were postoperative dysrhythmias and need for inotropes (p < or =0.01). Freedom from reoperation at 5, 10, 15 and 20 years was 86% (CI 78-95%), 69% (CI 56-85%), 53% (CI 38-74%) and 48% (CI 23-71%), respectively. Multivariate predictors for reoperation were preoperative mitral valve prolapse and an initial valve-sparing aortic procedure (p < or =0.05). In the composite valve conduit patients, freedom from thromboembolism was 88% (CI 76-100%), and from endocarditis was 99% (CI 93-100%) at 15 years. CONCLUSION: Composite valve conduit replacement of the aortic root in patients with Marfan syndrome offers a durable result, with low mortality and long-term complication rates. Reoperation was most commonly required for cardiac and vascular disease unrelated to the initial operation and in patients undergoing a valve-sparing aortic root procedure.
Outcomes of pregnancy in women with tetralogy of Fallot.
OBJECTIVES: We sought to determine pregnancy outcomes in patients with tetralogy of Fallot (TOF). BACKGROUND: Pregnancy outcomes in patients with TOF are incompletely defined. METHODS: Clinical, hemodynamic, and obstetric data were reviewed for women with TOF and prior pregnancy. RESULTS: Of 72 respondents, 43 (mean age, 26 years) had 112 pregnancies (range, 1 to 5); 82 pregnancies were successful. Eight women had unrepaired TOF at the time of their 20 successful pregnancies. At first assessment (age > or =18 years), six patients had pulmonary hypertension, three had moderate or severe right ventricular (RV) systolic dysfunction, and 13 had severe RV dilation due to pulmonic regurgitation. Sixteen patients had 30 miscarriages (27%) and one term stillbirth. Mean overall birth weight was 3.2 kg (range, 2.1 to 4.2 kg). Unrepaired TOF (p = 0.05) and morphologic pulmonary artery abnormality (p = 0.03) were independently predictive of infant birth weight. Six patients had cardiovascular complications during pregnancy: supraventricular tachycardia in two, heart failure in two, pulmonary embolism in a patient with pulmonary hypertension, and progressive RV dilation in a patient with severe pulmonic regurgitation. Five infants (6%) had congenital anomalies. CONCLUSIONS: Patients with TOF have an increased risk of fetal loss, and their offspring are more likely to have congenital anomalies than offspring in the general population. Adverse maternal events, although rare, may be associated with left ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with RV dysfunction.