Sabrina D. Phillips

Surgical Management of Anomalous Aortic Origin of a Coronary Artery

BACKGROUND Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus traveling between the aorta and pulmonary artery is associated with ischemia and sudden death. METHODS A retrospective review of 36 patients (23 male) who underwent operation between October 1992 and August 2008 for AAOCA was performed. Median age was 47 years (range, 13 to 82 years). Angina, shortness of breath, or syncope was present in 29 (81%), and 9 of 21 (43%) had an abnormal stress test. Coronary or computed tomographic angiography demonstrated an anomalous left main coronary artery arising from the right sinus in 13 (36%), right coronary artery arising from the left sinus in 21 (58%), and left anterior descending artery arising from the right sinus traveling between the aorta and pulmonary artery in 2 (5%). An intramural course was identified on preoperative imaging in 34 (94%). Although no patients had significant associated atherosclerotic coronary artery disease, 5 (14%) had previous acute myocardial infarction related to the AAOCA. RESULTS Operation included coronary artery bypass grafting in 14 patients and unroofing in 22; 6 patients had associated cardiac procedures performed. There were no early deaths. There was one late death secondary to a subdural bleed. At follow-up (mean 1.1 years; maximum 14 years), chest pain recurred in 1 patient who had coronary artery bypass grafting. No recurrent symptoms were noted in the unroofing group. CONCLUSIONS Unroofing of an anomalous coronary artery can be performed safely with excellent results in the majority of patients. When concomitant atherosclerotic coronary artery disease is present, coronary artery bypass grafting is an appropriate alternative.

Congenital heart disease and the liver.

There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug‐related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart‐liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long‐term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. (HEPATOLOGY 2012;56:1160–1169)

Cardiopulmonary Bypass During Pregnancy

BACKGROUND Cardiac surgery during pregnancy carries significant maternal and fetal risk and is typically considered after failure of medical therapy. We sought to determine the maternal and neonatal outcomes of cardiopulmonary bypass during pregnancy. METHODS Twenty-one pregnant patients undergoing cardiothoracic surgery were identified from the Mayo Clinic surgical database (1976 to 2009). Maternal and neonatal outcomes were reviewed. RESULTS Operations included 8 aortic valve replacements, 6 mitral valve repair-replacements, 2 myxoma excisions, 1 patent foramen ovale closure, 1 myectomy, 2 aortic aneurysm repairs, and 1 prosthetic aortic valve thrombectomy. Median cardiopulmonary bypass time was 53 minutes (range 16 to 185). Twelve patients (57%) required emergent surgery with a median gestational age (GA) of 25 weeks (range 7 to 35.5). Seven patients underwent cesarean section immediately prior to sternotomy delivering viable infants (median GA 31 weeks). In the remaining patients, three additional preterm births occurred, all in operations performed at an early GA (13 to 15 weeks). Median follow-up was 16 months (range 3 to 305). All patients improved to New York Heart Association functional class I or II. One early maternal death occurred 2 days after emergent mechanical aortic valve thrombectomy and 3 late maternal deaths occurred 2, 10, and 19 years postoperatively. Three fetal deaths occurred in mothers with additional medical comorbidities. CONCLUSIONS In the current era, cardiothoracic surgery can be performed with relative safety during pregnancy. Fetal complications (prematurity and death) are associated with urgent, high-risk surgery, maternal comorbidity, and early GA. Emergent surgery appears to confer a higher risk of maternal death.

Outcome of direct current cardioversion for atrial arrhythmias in adults with congenital heart disease

OBJECTIVES We sought to evaluate safety, efficacy, and outcome of direct current cardioversion (DCCV) for atrial arrhythmias in adults with congenital heart disease (CHD). BACKGROUND Atrial arrhythmias are increasingly noted in adults with CHD. The outcome of DCCV for atrial arrhythmias in this population is unknown. METHODS Our study was a retrospective review of patients 18 years or older with CHD who underwent DCCV between June 2000 and July 2003. This constituted the CHD group. Patient characteristics reviewed included the specific cardiac diagnosis and arrhythmia history. A subset of patients had transesophageal echocardiography (TEE) before DCCV; this subset was reviewed to evaluate spontaneous echocardiographic contrast. The outcome data evaluated included success of DCCV, complications, recurrence of arrhythmia, antiarrhythmic medication use, electrophysiology or pacemaker procedure in follow-up, and all-cause mortality. The recurrence rate of the arrhythmia was compared to a control group consisting of an age, gender, and rhythm matched group of patients who have no CHD and who underwent DCCV for atrial arrhythmias. RESULTS Sixty-three patients in the CHD group underwent 80 DCCVs, 59 of which were TEE-guided. Atrial flutter was more common in the CHD group (37 of 80 DCCV, 46%) than in the control group (13 of 56, 23%) (p<0.001). DCCV was successful in 75 (94%). Mean follow-up was 387 days. No thromboembolic events were noted. All-cause mortality on follow-up was 11%. There was no death related to DCCV. Twenty-five patients in the CHD group (40%) remained in sinus rhythm throughout follow-up. This was similar to that observed in the control group (30/56, 54%, p=0.13). Recurrent arrhythmia in the CHD group was predicted by the presence of atrial fibrillation (p=0.009) and less so spontaneous echo contrast in the left atrium (p=0.05). CONCLUSIONS DCCV with appropriate anticoagulation is safe and effective for patients with CHD, even in the presence of an intracardiac shunt and spontaneous contrast on TEE. However, the recurrence rate is substantial. Spontaneous echo contrast in the left atrium along with atrial fibrillation predicts arrhythmia recurrence following DCCV in patients with CHD.

Single-Center Experience With Implantable Cardioverter-Defibrillators in Adults With Complex Congenital Heart Disease

Adults with congenital heart disease are at risk of lethal ventricular arrhythmias and are candidates for implantable cardiac defibrillator (ICD) therapy, yet implant risks, long-term outcomes, and rates of appropriate and inappropriate ICD therapies are not well characterized. We reviewed clinical, implantation, and follow-up data on all transvenous ICDs in adults with congenital heart disease at the Mayo Clinic from 1991 through 2008. Seventy-three adults with congenital heart disease received 85 ICDs. Implantation diagnoses included tetralogy of Fallot (44%) and congenitally corrected transposition of the great arteries (17%). Implantation indication was occurrence of sustained ventricular arrhythmias (secondary prevention) in 36% and prophylactic (primary prevention) in the remainder. There were no major implant-related complications. During follow-up (2.2 ± 2.8 years, range 0 to 15) 11 patients died and 4 patients received heart or heart/lung transplants. An appropriate shock for a ventricular arrhythmia was observed in 19% of patients and an inappropriate shock was observed in 15% of patients. Likelihood of an appropriate shock was associated with increased subpulmonic ventricular pressure. In conclusion, implantation of transvenous ICDs in adults with congenital heart disease is associated with a low risk of implant complications. In this high-risk adult population the rate of inappropriate ICD shocks is low, whereas the likelihood of appropriate therapy for potentially lethal ventricular arrhythmias is high. These data suggest overall benefit of ICD therapy in adults with congenital heart disease.

Pregnancy and delivery in patients with fontan circulation: a case report and review of obstetric management.

The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of congenital heart disease that cannot support a biventricular circulation. An increasing number of women who have undergone these connections in childhood are now surviving into adulthood, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circulation pose a number of problems during pregnancy. Here, we report a case of a woman who underwent a Fontan procedure at age 7 and experienced significant cardiovascular decline before successfully delivering a viable infant at 33 weeks gestation. In addition, we reviewed the pertinent published data in this area, which suggests that pregnant patients with a Fontan circulation are more likely to face obstetrical, rather than cardiovascular, complications, including preterm labor, intrauterine growth restriction, an increased risk of cesarean section, and the potential need for anticoagulation. The review provides the obstetrician with the information needed to take a prominent role in the appropriate management of this rare, but growing, patient population. Target Audience: Obstetricians & Gynecologists, Family Physicians Leaning Objectives: After completion of this article, the reader will be able to describe the Fontan circulation, describe the importance of the collaborative practice model for patients with Fontan circulation, and identify potential complications in the pregnancy of women with Fontan circulation.

Contraception Practices and Pregnancy Outcome in Patients after Fontan Operation

OBJECTIVE The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation. DESIGN We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study. RESULTS Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect). CONCLUSIONS Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population.

Reoperation After Arterial Switch: A 27-Year Experience

BACKGROUND The long-term outcome and spectrum of reoperation after the arterial switch operation (ASO) has not been fully defined, and there are limited data in the literature. We reviewed our institutional experience with reoperation(s) after ASO. METHODS Between January 1984 and January 2012, 32 patients (23 male) underwent reoperation(s) after ASO. Anatomy included simple transposition of the great arteries in 14, complex transposition of the great arteries in 14, and Taussig-Bing in 4. Mean age was 6.7 ± 1.4 years at first operation and 10.8 ± 13.4 years at the second operation. Isolated pathology was present in 11 (34.3%) and multiple pathologies in 21 (65.6%). Abnormalities at first reoperation were right-sided pathology in 18 (56.3%), left-sided pathology in 10 (31%), coronary artery in 3 (9%), mitral valve in 3 (9%), residual ventricular septal defect in 4 (12.5%), and recoarctation in 2 (6.3%). It was the second reoperation in 12 and the third reoperation in 3 patients. RESULTS The first reoperation included pulmonary artery patch plasty in 18, aortic valve operation in 8 (4 valve replacement, 3 root replacement, and 1 repair), pulmonary valve replacement in 4, coronary artery bypass grafting in 3, and mitral valve repair in 3. Multiple reoperations occurred in 15 patients, comprising right-sided procedures (11), left-sided (2), and other (2). Pulmonary artery reconstruction occurred earlier than neoaortic intervention (5.4 ± 6.8 vs 13.8 ± 7.7 years, p < 0.001). There were 2 early deaths (6.2%); both patients had complex transposition of the great arteries and both were at early reoperation after ASO. Median follow-up was 14.5 years (maximum, 27 years). There were no late deaths. Freedom from reoperation at 1, 5, and 15 years was 88%, 78%, and 41%, respectively. CONCLUSIONS The most common indication for reoperation after ASO is right-sided pathology, followed by neoaortic root pathology. Late survival after ASO is excellent and risk of late reoperation is low. Life-long medical surveillance is required.

The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation

BACKGROUND Approximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described. METHODS Seven patients with refractory PLE after the Fontan operation were started on oral CR-budesonide at 9 mg. After 3 to 9 months, the dose was weaned to 3 mg. Response to treatment was assessed by clinical evaluation, serum albumin levels, and fecal α-1 antitrypsin clearance when available. RESULTS Median age at last evaluation was 20 years (range, 16 to 32 years). Six patients had increases in serum albumin levels but only 4 patients had symptomatic improvement. Systemic side effects included: cushingoid features (5), adrenal insufficiency (4), and new-onset type 2 diabetes mellitus (2). One patient had improvement in cushingoid features after weaning CR-budesonide to 3 mg. Older patients (ages 27 to 32 years) had the worst side effect profiles and were the most refractory to treatment. These patients had sonographic evidence of hepatic cirrhosis but normal serum liver function tests. Two deaths occurred: 1 from sepsis 1 month after CR-budesonide initiation and 1 from respiratory arrest 5 months after CR-budesonide discontinuation. CONCLUSIONS CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well.

Ventricular Arrhythmia Risk Stratification in Patients With Tetralogy of Fallot at the Time of Pulmonary Valve Replacement

Background—Most patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR), but the evaluation for and management of ventricular arrhythmia remain unclear. This study is aimed at clarifying the optimal approach to this potentially life-threatening issue at the time of PVR. Methods and Results—A retrospective analysis was performed on 205 patients with repaired tetralogy of Fallot undergoing PVR at our institution between 1988 and 2010. Median age was 32.9 (range, 25.6) years. Previous ventricular tachycardia occurred in 16 patients (8%) and 37 (16%) had left ventricular dysfunction, defined as left ventricular ejection fraction <50%. Surgical right ventricular outflow tract cryoablation was performed in 22 patients (10.7%). The primary outcome was a combined event including ventricular tachycardia, out-of-hospital cardiac arrest, appropriate implantable cardioverter defibrillator therapy, and sudden cardiac death. Freedom from the combined event at 5, 10, and 15 years was 95%, 90%, and 79%, respectively. In the first year after PVR, 2 events occurred. Conversely, in the 22 patients who underwent surgical cryoablation, a single event occurred 7 years after PVR. A history of ventricular tachycardia and left ventricular dysfunction was associated with higher risk for the combined event (hazard ratio, 4.7; P=0.004 and hazard ratio, 0.8; P=0.02, respectively). Conclusions—Patients with repaired tetralogy of Fallot undergoing PVR with history of ventricular tachycardia or left ventricular dysfunction appear to be associated with a higher risk of arrhythmic events after operation. Events in the first year after PVR are rare, and in select high-risk patients, surgical cryoablation does not seem to increase arrhythmic events and may be protective.