Cardiovascular Disease Screening in Women: Leveraging Artificial Intelligence and Digital Tools.

Cardiovascular disease remains the leading cause of death in women. Given accumulating evidence on sex- and gender-based differences in cardiovascular disease development and outcomes, the need for more effective approaches to screening for risk factors and phenotypes in women is ever urgent. Public health surveillance and health care delivery systems now continuously generate massive amounts of data that could be leveraged to enable both screening of cardiovascular risk and implementation of tailored preventive interventions across a woman's life span. However, health care providers, clinical guidelines committees, and health policy experts are not yet sufficiently equipped to optimize the collection of data on women, use or interpret these data, or develop approaches to targeting interventions. Therefore, we provide a broad overview of the key opportunities for cardiovascular screening in women while highlighting the potential applications of artificial intelligence along with digital technologies and tools.

Effect of Earlier Atrioventricular Valve Intervention on Survival After the Fontan Operation.

Whereas the prevalence and impact of atrioventricular valve (AVV) regurgitation in patients with single ventricle physiology has become increasingly apparent, the optimal timing for valve intervention is unclear. To investigate this, we performed a retrospective review of all 1,167 patients from the Mayo Clinic Fontan database. Thirteen percent (153 patients) had AVV repair or replacement during their staged single ventricle palliation. We found that patients with right ventricular morphology and common AVV were at increased risk for AVV intervention. Patients who underwent AVV intervention had increased risk of death/transplant compared with those who did not (hazards ratio [HR] = 1.75, 95% CI 1.37 to 2.23, p <0.001). With respect to valve intervention timing, whereas AVV intervention before Fontan presented similar risk for death/transplant compared with no AVV intervention (HR = 0.85, 95% CI 0.32 to 2.27, p = 0.74), intervention at time of Fontan had a significantly higher risk (HR = 1.46, 95% CI 1.09 to 1.97, p = 0.01), and intervention after Fontan had a much more substantial risk (HR = 3.83, 95% CI 2.54 to 5.79, p <0.001). AVV repair failure occurred in 11% of patients. In terms of relative risk of valve repair versus replacement, in post-Fontan AVV intervention patients, AVV replacement carried a 2.9 fold risk of death/transplant compared with AVV repair. In conclusion, AVV disease remains a considerable challenge for durable Fontan physiology. This data demonstrates that earlier intervention on valve pathology improves survival with the Fontan circulation. Continued surveillance of single ventricle patients and prompt referral of those with valve pathology can improve outcomes in this challenging population.

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.

Surgical unroofing of anomalous aortic origin of a coronary artery: a single-center experience.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The optimal management of patients with AAOCA is controversial. We examined our experience with surgical unroofing of AAOCA to determine the midterm effect of surgical repair. METHODS: From October 1992 through December 2011, 75 patients with AAOCA underwent surgical unroofing. RESULTS: Mean age was 39.6 ± 19.6 years; 23 patients (32%) were aged younger than 30 years. Angina, shortness of breath, or syncope was present in 55 patients (72%); 2 (3%) had history of sudden cardiac arrest. Of 40 patients (53%) who had preoperative stress tests, results were abnormal in 20 (50%). Coronary or computed tomography angiography demonstrated an anomalous right coronary artery (RCA) arising from the left sinus in 69 patients (92%) and the left main coronary artery arising from the right sinus in 6 (8%). Two patents (3%) were referred for recurrent anginal symptoms after previous RCA bypass with the right internal mammary artery. Minimally invasive partial upper sternal split was performed in 17 patients (22%). Two patients (3%) needed right internal mammary artery-to-RCA grafting due to flow acceleration at the RCA ostium. There were no early deaths. One late death (1%) occurred related to noncardiac causes. At follow-up (mean, 18 months; maximum, 7 years), all patients remained free of cardiac symptoms. CONCLUSIONS: Surgical unroofing of AAOCA is associated with low morbidity and mortality. At intermediate follow-up, resolution of symptoms and freedom from sudden death can be expected. The threshold for offering intervention should be low.

Should repair of partial atrioventricular septal defect be delayed until later in childhood?

Surgical repair of partial atrioventricular septal defects (AVSD) has been successful for more than 60 years. However, recent data from the Pediatric Heart Network show that 31% of patients have moderate or severe left atrioventricular valve regurgitation (LAVVR) at follow-up. Previously, our institution found that only 9% of patients had more than moderate LAVVR at the last follow-up. Our objective was to determine the long-term outcomes after repair of partial AVSD in the current era. We reviewed all patients with partial AVSD who had primary biventricular repair from January 1995 to June 2011 at our institution. The Kaplan-Meier method was used to estimate the survival free of an event, and factors were evaluated for an association with each outcome using the log-rank test. All 105 patients with partial AVSD who had surgery during this period were evaluated. The median age at surgery was 7.9 years. The overall survival rate at 1 year was 97%. Median follow-up was 5.3 years (interquartile range 1.7 to 11.1). At 3 years, the survival rate free from reoperation was 89%. Thirteen patients required reoperations with the most common reason being LAVVR. A total of 10 patients developed more than moderate LAVVR with a cumulative incidence of 8% by 2 years. The discrepancy with the Pediatric Heart Network data may be due to the later age of operation for patients in our cohort suggesting that elective repair of partial AVSD should be deferred until children are somewhat older (ages 5 to 8 years). Neither patient age (p = 0.11) nor severity of preoperative LAVVR (p = 0.16) were identified as statistically significant risk factors. In conclusion, there is less morbidity and mortality after surgical repair for partial AVSD.

Right ventricular unloading for heart failure related to Ebstein malformation.

BACKGROUND: Patients with Ebstein malformation (EM) and severe RV dilatation and dysfunction have increased operative risk. Early results with right ventricular unloading utilizing the bidirectional cavopulmonary shunt (BCPS) during repair of EM have been encouraging. We report our experience of the 1.5-ventricle repair strategy for this difficult group of patients. METHODS: Between July 1999 and January 2013, 62 patients with severe EM underwent BCPS at the time of tricuspid repair. Median age was 21.5 years (range, 9 months to 57 years), 51.6% were male, and 72.5% were children. Severe RV dilatation was present in all patients; severe RV dysfunction was present in 72.5% (n=45) and moderate to severe RV dysfunction in 22.5% (n=14). Mean RV systolic pressure was 32.7±0.7 mm Hg and mean PA pressure was 15.6±2.1 mm Hg. Mean preoperative left ventricular ejection fraction (LVEF) was 0.536±0.071; it was less than 40% in 10 patients (16.1%). New York Heart Association class III/IV heart failure was present in 43 patients (69.3%) preoperatively and 20 patients (32.2%) were initially referred for heart transplant evaluation. Prior EM surgery occurred in 35.4% (n=22; 8 prior valve repair, 8 prior valve replacement, Blalock-Taussig shunt in 4, atrial septal defect (ASD) closure in 2). RESULTS: Tricuspid repair was performed in 51.6% (n=32, 5 had re-repair). Bioprosthetic valve replacement was performed in 48.4% (n=30, 8 had rereplacement). The BCPS was a planned procedure in 53 patients (85.5%) because of RV dysfunction; BCPS was added after unsuccessful weaning from bypass in 7 (11.2%), and in the early postoperative period due to hemodynamic instability in 2. Concomitant procedures included ASD closure in 48.3%, maze in 38.7%, and mitral valve repair in 6.4%. Postoperative extracorporeal membrane oxygenation support was needed in 8 patients. Delayed chest closure was performed in 25.8%. Early mortality was 1.6% (n=1). Mean mechanical ventilation time was 69.7 hours. Mean intensive care unit and hospital stays were 5.4±3.5 and 10.7±3.5 days, respectively. Mean follow-up was 3.6±2.6 years (maximum, 12.8 years). Patients (n=10) with low preoperative LVEF (0.362±0.035) improved to 0.517±0.042 postoperatively (p=0.001). There was 1 late death in a patient with cystic fibrosis. Late reintervention was needed in 5 patients (8%). Late follow-up was available in 95% (n=59); all were acyanotic and 88% were in New York Heart Association functional class I/II. CONCLUSIONS: Concomitant BCPS is a useful adjunct in repair of advanced EM with severe RV dilatation and dysfunction. Operation can be performed with low early mortality. Intermediate-term survival and quality of life is good to excellent, and transplantation can be delayed or avoided in the majority.

Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults.

OBJECTIVES: Limited data are available regarding the surgical strategies for an anomalous origin of the coronary artery from the pulmonary artery (ACAPA) in adulthood. We reviewed our surgical experience with ACAPA in adults. METHODS: From January 1960 to July 2011, 10 adults (30% men), aged 18 to 78 years (median, 43), underwent surgical repair of ACAPA. Anomalous left (ALCAPA) was present in 7, anomalous right (ARCAPA) in 2, and anomalous left anterior descending artery in 1. Most (90%) were symptomatic, with 7 (70%) having ischemic changes on a preoperative at rest or stress electrocardiogram. One patient had undergone previous ligation of ALCAPA in childhood. The mean left ventricular ejection fraction was 56% ± 10%, with mild dysfunction in 4. RESULTS: Of the 10 patients, 7 underwent repair by coronary artery button transfer to the aorta, with 2 requiring an interposition saphenous vein graft. In 3 patients, coronary transfer was not possible, and coronary artery bypass grafting with closure of the ACAPA was performed. The mean follow-up was 8.6 years (maximum, 37). Two late deaths occurred from noncardiac causes. One patient with ALCAPA who underwent direct reimplantation subsequently required coronary artery bypass grafting for left main stenosis, likely secondary to tension. At the last follow-up visit, all patients were in New York Heart Association class I or II. The postoperative left ventricular ejection fraction was similar at 53% ± 9%; No significant improvement was seen in those with below normal ejection fraction. CONCLUSIONS: Establishment of a dual coronary artery system with coronary transfer is preferred, even if an interposition graft is required. Coronary artery bypass grafting with closure of ACAPA should be reserved for when coronary transfer is not feasible. Surgical correction of ACAPA should be considered before the onset of left ventricular dysfunction.

Adults with congenital heart disease and previous intervention.

Cardiac malformations occur in approximately 1% of live births. Advances in surgery, interventional cardiology, and medical care have translated into increasing numbers of adult patients with congenital heart disease. These patients, even after intervention, have cardiac sequelae that require specialized care by cardiologists and cardiac surgeons with expertise in the management of congenital cardiac disease. We review 8 of the most common lesions encountered after intervention in an adult congenital cardiac practice and discuss longitudinal follow-up, with a focus on appropriate testing, common hemodynamic and electrophysiological issues, and indications for reintervention.

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.

Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis.

OBJECTIVE: We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease. METHODS: From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis. RESULTS: The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P < .003) and portal fibrosis (P < .002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P < .03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome. CONCLUSIONS: The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome.

Late outcome of repair of congenital coronary artery fistulas--a word of caution.

OBJECTIVES: We reviewed our experience with repair of congenital coronary artery fistulas. METHODS: From June 1983 to December 2009, 46 patients (median age, 59 years; range, 1-84 years) underwent surgical repair. The presenting symptoms included angina in 16 patients (35%), congestive heart failure in 11 (24%), and bacterial endocarditis in 5 (11%). Preoperatively, 9 patients (20%) had at least moderate tricuspid regurgitation. Coronary artery dominance was right in 38 patients (83%). Coronary artery aneurysms were found in 8 patients (17%). The most common pattern was right coronary artery-to-coronary sinus fistula (18 patients, 39%); 11 patients had (23%) more than 1 fistula. One patient had undergone previous coil embolization. RESULTS: Cardiopulmonary bypass was used in 39 patients (85%), with extracardiac and intracardiac repair performed in 30 (65%) and 16 (35%), respectively. The most common associated procedures were coronary artery bypass in 13 patients (28%). Early mortality occurred in 1 patient (2%). Postoperative myocardial infarction occurred in 5 patients (11%); 4 of these patients underwent simple ligation or division of their fistulas. The mean follow-up was 6 ± 5.8 years (maximum, 22 years). Late mortality occurred in 11 patients (24%). Two patients underwent reoperation for severe tricuspid regurgitation. Survival was significantly reduced compared with the age- and gender-matched population (P = .03). Residual fistulas were detected in 3 patients (6%), with no reintervention needed. CONCLUSIONS: Perioperative myocardial infarction is an important complication of ligation of coronary artery fistulas and can contribute to reduced late survival. The tricuspid valve should be evaluated carefully at repair because of the relatively high rate of residual regurgitation in survivors.

Fontan conversion to one and one half ventricle repair.

BACKGROUND: In patients with a modified Fontan connection, particularly the right atrial to right ventricular connection, the pulmonary ventricle may enlarge with time. METHODS: Between January 1990 and December 2006, 10 patients (median age, 24 years) underwent Fontan conversion to a one and one half ventricle repair. Tricuspid atresia was the most common diagnosis (n=7). The right atrial to right ventricular connection was present in 8 patients; 3 patients had a prior bidirectional cavopulmonary anastomosis. Preoperative median right atrial pressure was 14 mm Hg (range, 12 to 20 mm Hg). Indications for surgery were exercise intolerance, arrhythmias, and conduit obstruction. Echocardiography showed moderate right ventricular hypoplasia, with right atrial to right ventricular regurgitation. The conversion included closure of septal defects, tricuspid valve replacement (n=8), bidirectional cavopulmonary anastomosis (n=7), valved right ventricular outflow tract reconstruction (n=2), and arrhythmia surgery (n=6). RESULTS: There was no early mortality. There was one reoperation for residual ventricular septal defect. Prolonged chest tube drainage occurred in 2 patients. Postoperative right atrial pressure ranged from 8 to 14 mm Hg (median, 10 mm Hg; p=0.02). Median follow-up was 8 years. There was no late mortality. Nine patients are in New York Heart Association class I or II, and 1 patient has biventricular failure and was awaiting transplantation. There were two late reoperations, 1 for thrombosis of the mechanical tricuspid valve prosthesis and 1 for obstruction of the valved right ventricular outflow tract conduit. Three patients had recurrent atrial arrhythmias requiring cardioversion. CONCLUSIONS: Fontan conversion to one and one half ventricle repair is feasible in selected patients with a failing Fontan circulation. Operation can be performed with low early mortality. Arrhythmia surgery should be performed routinely. Quality of life is excellent.

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.