The Value of the Electrocardiogram in Adult Congenital Heart Disease.

The electrocardiogram is the first test that is undertaken when evaluating a patient's heart. Diagnosing congenital heart disease in an adult (ACHD) can be facilitated by knowing the classical electrocardiographic (EKG) findings. These EKG findings often result from the congenital defect that prevents a part of the cardiac conduction system from occupying its normal anatomic position. When these classical EKG findings are not present, the clinician should consider alternate diagnoses. As the patient with congenital heart disease ages, with native anatomy or after surgical or device repair, the EKG can be used to assess the patient's status and to decide if and when treatment requires adjustment. This is because the electrocardiogram (EKG) can diagnose the hypertrophy or enlargement in a cardiac chamber that results from the congenital defect or anomaly and can diagnose an arrhythmia that might compromise an otherwise stable anatomy. While ACHD often involves intracardiac shunting, in many cases the abnormality only involves cardiac electrical conduction block or ventricular repolarization. These life-threatening diseases can be diagnosed with an EKG. This review will demonstrate and explain how the EKG can be used to diagnose and follow adults with congenital heart disease. When coupled with history and physical examination, the value of the EKG in ACHD will be apparent. A diagnosis can then be made or a differential diagnosis proposed, before an imaging study is ordered.

Chest Radiography Pearls in Select Adult Congenital Heart Disease.

Congenital heart disease in adult patients (ACHD) includes individuals with native anatomic deformities and those who have benefited from corrective, ameliorative, or interventional heart and vascular interventions. Congenital heart disease is the most common birth defect, although with interventions most survive into adulthood. Newborns and children with complex congenital heart diseases that feature cyanosis fail to thrive, and once this is identified, heart failure can promptly undergo diagnostic evaluations and treatment. However, patients with simple congenital heart disease and subtle clinical signs and symptoms may escape diagnosis until adulthood or experience changes in their cardiac hemodynamics and physiology in settings such as pregnancy or newly diagnosed arrhythmias. The chest X-ray (CXR) is the most common X-ray among all radiological procedures. Individual features or a constellation of features on a CXR are often present in patients who have congenital heart disease. The ability to recognize these CXR features is a valuable skill for making the diagnosis of ACHD and for following these patients as they age, and can complement echocardiographic findings. When used well to diagnose ACHD, the CXR will be the sharpest arrow in the quiver.

Adult Congenital Heart Disease in the Emergency Department.

While congenital heart disease historically was a pathology primarily restricted to specialized pediatric centers, advances in technology have dramatically increased the number of people living into adulthood, the number of complications faced by these patients, and the number of patients visiting non-specialized emergency departments for these concerns. Clinicians need to be aware of the issues specific to patients' individual congenital defects but also have an understanding of how typical cardiac pathology may manifest in this special group of patients. This manuscript attempts to provide an overview of this diverse but increasingly common group of adult patients with congenital heart diseases, including a review of their anatomical variants, the complications they face at the highest rates, and ways that emergency physicians may need to manage these patients differently to avoid causing harm.

Safety and Efficacy of Surgical and Percutaneous Cardiac Interventions for Adults With Down Syndrome.

Objective: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). Patients and Methods: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic. Results: In total, 81 adults (43 men; 38 women) with DS underwent 89 cardiac interventions (84 surgical; 5 percutaneous) at a mean age of 33 years. Twenty-six patients presented with complete atrioventricular canal defect (17%) or tetralogy of Fallot (15%). The most common adult procedures were valve interventions: mitral (31%), tricuspid (15%), and pulmonary (12%). Of pulmonary valve interventions in the new cohort, 33% were performed percutaneously. The postoperative mortality rate was low (1% total). The mean time between last operation and death was 16 years. Conclusion: Adults with DS can undergo cardiac operation and percutaneous intervention with low morbidity and mortality risk and good long-term survival.

Anomalous Left Coronary Artery from the Pulmonary Artery: How to Diagnose and Treat.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly that can present in childhood or adulthood with a multitude of symptoms depending on the age of presentation. It should be suspected in infants presenting with heart failure in the setting of left ventricular systolic dysfunction and associated mitral regurgitation from papillary muscle ischemia. Adults with ALCAPA may present with cardiac ischemic symptoms. Prompt diagnosis with echocardiography and cross-sectional chest imaging is important to guide surgical intervention and improve the patients' survival and prognosis. The goal of surgery is to establish a dual-coronary system with mid-term results revealing progressive recovery of left ventricular function and improvement in mitral regurgitation. Patients with ALCAPA should maintain life-long follow-up with a cardiologist with congenital heart disease expertise for surveillance of post-operative complications.

Congenital Heart Disease: A Growing Population with Challenges to Patients and Providers.

Congenital heart disease (CHD) is the most frequent disorder encountered in the newborn period and the most frequent cause of infant mortality [...].

Left ventricular outflow tract obstruction in patients with Ebstein anomaly.

Objective: In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible mechanisms of obstruction as assessed by 2-dimensional echocardiography. Methods: We queried our institutional echocardiography and surgical databases for patients with the diagnosis of Ebstein anomaly seen from 1985 through 2022. Fourteen patients had the additional diagnosis of LVOT obstruction identified from transthoracic echocardiography. Results: The mean age of the 14 patients at the time that LVOT obstruction was identified was 51.9 ± 9.9 years, and 11 (78%) were female. LVOT obstruction (mean gradient 61.0 ± 25.7 mm Hg) was documented at the time of initial diagnosis of Ebstein anomaly for 8, and in the remaining, was recognized on follow-up imaging. Dynamic LVOT obstruction with systolic anterior motion (SAM) was the most common mechanism and was mostly accompanied by leftward septal movement and septal hypertrophy. Seven of the 9 patients with SAM had the diagnosis of hypertrophic cardiomyopathy. Leftward bowing of the septum appeared to be the sole cause of LVOT obstruction in 3 and a contributing factor to SAM in 4. Among the 13 patients who underwent an operation for Ebstein anomaly, 7 had concomitant septal myectomy and 3 had a subsequent procedure. Conclusions: There are multiple mechanisms of LVOT obstruction in patients with Ebstein anomaly, including SAM and leftward bowing of the interventricular septum, which may exist alone or in combination. Septal myectomy at the time of tricuspid valvuloplasty is safe and effectively reduces gradients in the outflow tract and may prevent the need for subsequent reintervention.

Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease.

Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.

Cardiopulmonary Outcomes After the Nuss Procedure in Pectus Excavatum.

Background Pectus excavatum is the most common chest wall deformity. There is still controversy about cardiopulmonary limitations of this disease and benefits of surgical repair. This study evaluates the impact of pectus excavatum on the cardiopulmonary function of adult patients before and after a modified minimally invasive repair. Methods and Results In this retrospective cohort study, an electronic database was used to identify consecutive adult (aged ≥18 years) patients who underwent cardiopulmonary exercise testing before and after primary pectus excavatum repair at Mayo Clinic Arizona from 2011 to 2020. In total, 392 patients underwent preoperative cardiopulmonary exercise testing; abnormal oxygen consumption results were present in 68% of patients. Among them, 130 patients (68% men, mean age, 32.4±10.0 years) had post-repair evaluations. Post-repair tests were performed immediately before bar removal with a mean time between repair and post-repair testing of 3.4±0.7 years (range, 2.5-7.0). A significant improvement in cardiopulmonary outcomes (P<0.001 for all the comparisons) was seen in the post-repair evaluations, including an increase in maximum, and predicted rate of oxygen consumption, oxygen pulse, oxygen consumption at anaerobic threshold, and maximal ventilation. In a subanalysis of 39 patients who also underwent intraoperative transesophageal echocardiography at repair and at bar removal, a significant increase in right ventricle stroke volume was found (P<0.001). Conclusions Consistent improvements in cardiopulmonary function were seen for pectus excavatum adult patients undergoing surgery. These results strongly support the existence of adverse cardiopulmonary consequences from this disease as well as the benefits of surgical repair.

Early and mid-term results of autograft rescue by Ross reversal: A one-valve disease need not become a two-valve disease.

OBJECTIVES: Risk of reoperation and loss of a second native valve are major drawbacks of the Ross operation. Rather than discarding the failed autograft, it can be placed back into the native pulmonary position by "Ross reversal." We review our early and mid-term results with this operation. METHODS: From 2006 to 2017, 39 patients underwent reoperation for autograft dysfunction. The autograft was successfully rescued in 35 patients: by Ross reversal in 30, David procedure in 4, and autograft repair in 1. Medical records were reviewed for patient characteristics (mean age was 46 ± 13 years, range 18-67 years, and 23 were male), previous operations, indications for reoperation, hospital outcomes, and echocardiographic findings for the 30 patients undergoing successful Ross reversal. Follow-up was 4.1 ± 3.5 years (range 7 months-11 years). RESULTS: Median interval between the original Ross procedure and Ross reversal was 12 years (range 5-19 years). Eight patients also had absolute indications for replacement of the pulmonary allograft. There was no operative mortality. One patient required reoperation for bleeding. Another had an abdominal aorta injury from use of an endoballoon clamp. There was no other major postoperative morbidity, and median postoperative hospital stay was 7.2 days (range 4-41 days). No patient required reoperation during follow-up. Twenty-four patients had acceptable pulmonary valve function, and 6 had clinically well-tolerated moderate or severe pulmonary regurgitation. CONCLUSIONS: Ross reversal can be performed with low morbidity and acceptable pulmonary valve function, reducing patient risk of losing 2 native valves when the autograft fails in the aortic position.

Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience.

OBJECTIVE: We sought to review our experience with isolated partial anomalous pulmonary veins as their management is controversial. DESIGN: We reviewed all patients with isolated partial anomalous pulmonary veins >18 years attending the Congenital Clinic from 1988-2008. RESULTS: Forty-three patients aged 20-73 years were included. Fifteen patients had no surgery, 11 of whom had a single anomalous pulmonary vein. Surgical repair was performed in 28 patients: 27 had right ventricular volume overload, 21 had more than one anomalous pulmonary vein, and 1 had stenosed pulmonary veins. Right ventricular systolic pressure was elevated (>35 mm Hg) in 12 patients. Eleven patients had anomalous left pulmonary veins: 10 had the pulmonary vein anastomosed to the left atrial appendage and one patient had anastomosis to the left lower pulmonary vein. One patient had bilateral anomalous pulmonary veins anastomosed directly to the left atrium. Sixteen patients had anomalous right pulmonary veins: fifteen had a baffle through a surgically-created atrial septal defect, while one had the anomalous pulmonary vein anastomosed to the left atrium. There was no early mortality. During mean echo follow-up of 2.7 years, 19 patients (68%) had reduced right ventricular size and five had improved right ventricular function. Seven patients with increased pulmonary pressure had a decrease post-operatively. CONCLUSION: Partial anomalous pulmonary veins can cause symptoms, right ventricular volume overload, and pulmonary hypertension, particularly when more than one pulmonary vein is anomalous. Surgical repair can be accomplished with low morbidity and mortality, with improvement in the right ventricular size and pulmonary pressures in most.

Adults with Down syndrome: safety and long-term outcome of cardiac operation.

OBJECTIVE: As limited data exist, we sought to review the safety and outcome of cardiac surgery in the adult Down syndrome population. DESIGN: We reviewed the data of all patients >or=18 years old with Down syndrome who underwent cardiac surgery (1969-2008) at our hospital. RESULTS: Fifty patients underwent 57 surgeries (mean age 33 years). Fifteen patients had prior cardiac operations in childhood: 7 complete and 1 partial atrioventricular canal, 2 secundum atrial septal defect, 2 Tetralogy of Fallot, 1 patent ductus arteriosus, 1 combined atrioventricular canal and Tetralogy of Fallot, and 1 ventricular septal defect. Operations in adult Down syndrome patients included repair of partial atrioventricular canal in 17, aortic valve replacement in 7, mitral valve replacement/repair in 7, ventricular septal defect in 6, atrial septal defect in 3, Tetralogy of Fallot in 3, pulmonary valve replacement in 3, and other in 11. There was 1 in-hospital death (1.8%) from multiorgan failure. The mean hospital stay was 10.6 days. Average ventilatory support was 2.4 days (range 0-32). Atrial arrhythmias occurred in 14 patients (25%). Six patients had early postoperative pulmonary infections. Mean follow-up was 6 years, maximum 29 years. There were eight late deaths at an average age of 52 years (range 30-58) occurring 15 years postoperatively (range 32 days-29 years); two in the setting of dementia (mean age 56 years). CONCLUSION: At an experienced center, adult patients with Down syndrome can undergo cardiac surgery with a low risk of mortality and acceptable morbidity. Atrial arrhythmias and pulmonary infections are common postoperatively.