Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country

BACKGROUND: The number of patients with congenital cardiac disease reaching adulthood is increasing steadily. Many adults with such disease face both medical and surgical difficulties. HYPOTHESIS: This retrospective study was undertaken to assess the frequency and outcome of congenital heart disease (CHD) in unoperated adults. METHODS: The charts of all patients with unoperated CHD, who were admitted to a tertiary care center in Beirut, Lebanon, between 1980 and 2000 were reviewed. Of these, 206 patients (52% men, age at admittance 18-71 years [32.8 +/- 13.3 years]) with a diagnosis of CHD were evaluated. Atrial septal defect (ASD) was the most common cardiac malformation with a relative frequency of 53%, followed by ventricular septal defects (11%), tetralogy of Fallot (11%), aortic anomalies (7%), pulmonary stenosis (6%), and Ebstein anomaly (4%). Most patients were symptomatic upon presentation, with dyspnea on exertion being the most common presenting symptom. Twenty-seven patients (13%) had cyanotic CHD. Of 179 acyanotic patients, 113 (63%), and 17 of 27 cyanotic patients (63%) underwent surgical intervention. In-hospital surgical complications for the acyanotic group included cerebrovascular accident (2%) and heart block (1%). Total surgical mortality was 4 of 130 (3%). One patient with tetralogy of Fallot presented with endocarditis and died. CONCLUSION: Atrial septal defect is the most common defect reported in our experience; however, it occurs more frequently than that reported in the literature. Although most patients were symptomatic on presentation, their functional status was stable. Accordingly, their hospital course, whether managed medically or surgically, held a relatively low complication rate. This could be attributed to the uncomplicated nature of pathologies in our series. The surgical mortality and in-hospital complications were slighter higher than those reported for similar lesions if repaired during childhood. This study reflects the relative frequency of various cardiac malformations in selected patients with "grown up" congenital heart disease (GUCH) and their natural survival pattern.     

Coronary artery disease screening in adults with congenital heart disease prior to cardiac surgery

Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart dis‐ ease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017.
Outcome Measures: Data regarding CAD risk factors and preoperative CAD screen‐ ing results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes.
Results: A total of 73 patients underwent CAD screening with either cardiac catheteri‐ zation (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD.
Conclusion: CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary inter‐ vention is uncommon. Preoperative CAD screening should be based on age and tra‐ ditional CAD risk factors, rather than underlying CHD.     

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery

Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty‐two patients (25%), experi‐ enced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty‐eight patients (15%) endured complication related early mor‐ bidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86‐0.98) for in‐hospital mortality; and 0.82 (95% CI: 0.76‐0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of compos‐ ite adverse outcome (OR: 14.2, 95% CI: 7.2‐28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7‐42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4‐41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4‐15.5; P < 0.0001).
Conclusions: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.     

Transplantation in adults with congenital heart disease

Considerable progress in pediatric cardiac surgery has led to more patients with congenital heart disease surviving into adulthood. However, progressive cardiopulmonary dysfunction often occurs late after palliative or corrective surgeries to the point where transplantation becomes the only treatment option. Adult congenital heart disease represents a growing population of patients being referred for heart, lung, and combined heart-lung transplantation. This group of patients presents multiple unique surgical and medical challenges to transplantation owing to their complex anatomy, multiple prior palliative and corrective procedures, frequently increased pulmonary vascular resistance, and often debilitated condition. Consequently, transplantation in adults with congenital heart disease is associated with a relatively high operative mortality secondary to increased bleeding, infection, and graft failure rates compared with noncongenital heart disease transplant recipients. However, those who survive of the first posttransplant year enjoy an excellent long-term prognosis.     

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.     

Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors

Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.
Design: Retrospective analysis.
Setting: Single center, quaternary care children’s hospital.
Patients: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.
Interventions: None.
Outcome Measures: The primary outcome was early postoperative atrial fibrillation. Secondary outcomes included preoperative comorbidities, preoperative echocardiographic findings, operative details, and postoperative morbidities, such length of stay, reintubation, stroke, and death.
Results: The incidence of early postoperative atrial fibrillation was 21%. Those who developed early postoperative atrial fibrillation were older (50 years vs 38 years, P =< .001), had a history of atrial fibrillation prior to surgery, had preoperative pulmonary hypertension, and had longer cardiopulmonary bypass times (103 minutes vs 84 minutes, P = .025) when compared to those who did not develop postoperative atrial fibrillation. Multivariate analysis identified age greater than 60, preoperative pulmonary hypertension, mitral valve intervention, and the need for postoperative inotropic support as being independent predictors of postoperative atrial fibrillation. Those who developed postoperative atrial fibrillation remained in the hospital longer (9 days vs 7 days, P =< .001).
Conclusions: Atrial fibrillation is a common complication following cardiac surgery in adults with congenital heart disease. Age, preoperative comorbidities, type of surgical intervention, and the need for perioperative inotropic infusions may predict the risk of atrial fibrillation in this unique patient population.     

Neurocognitive profiles in adolescents and young adults with congenital heart disease

Introduction and Objectives

The objectives of this study were to assess the neuropsychological performance (NP) of adolescents and young adults with congenital heart disease (CHD), comparing them with a group of healthy controls, to determine whether there are different neurocognitive phenotypes in CHD, and to identify their relation to sociodemographic, neonatal, clinical and psychological adjustment variables.

Indications for heart transplantation in congenital heart disease

In this review we have looked at indications for cardiac transplantation in congenital heart disease. An outline of the general principles of the use of transplant as a management strategy both as a first line treatment and following other surgical interventions is discussed. We explore the importance of the timing of patient referral and the evaluations undertaken, and how the results of these may vary between patients with congenital heart disease and patients with other causes of end-stage heart failure. The potential complications associated with patients with congenital heart disease need to be both anticipated and managed appropriately by an experienced team. Timing of transplantation in congenital heart disease is difficult to standardize as the group of patients is heterogeneous. We discuss the role and limitations of investigations such as BNP, 6 minute walk, metabolic exercise testing and self estimated physical functioning. We also discuss the suitability for listing. It is clear that congenital heart patients should not be considered to be at uniform high risk of death at transplant. Morbidity varies greatly in the congenital patient population with the failing Fontan circulation having a far higher risk than a failing Mustard circulation. However the underlying issue of imbalance between donor organ supply and demand needs to be addressed as transplant teams are finding themselves in the increasingly difficult situation of supporting growing numbers of patients with a diverse range of pathologies with declining numbers of donor organs.     

Echocardiography in adults with congenital heart disease

Echocardiography is a valuable tool in the investigation and follow-up of adult patients with congenital heart disease. The majority of these patients have been recognised and treated in childhood, and effective investigations in adult life depend on exact knowledge of the patient history and previous treatments/operations. The major lesions are presented and important echocardiographic features briefly discussed.     

右美托咪定对小儿先天性心脏病外科手术麻醉血流动力学的影响

目的探讨右美托咪定对小儿先天性心脏病外科手术麻醉过程中血流动力学的影响。方法将68例在体外循环下行心脏手术的先天性心脏病患儿按随机数字法随机分为咪达唑仑组(n=34)和右美托咪定组(n=34)。麻醉诱导:两组均给予咪达唑仑0.2 mg/kg、芬太尼10μg/kg、维库溴铵0.2 mg/kg行麻醉诱导。麻醉诱导后,行气管内插管,机械通气。麻醉维持:咪达唑仑组输注咪达唑仑0.2 mg.kg-1.h-1和芬太尼10μg.kg-1.h-1,1 h后分别以0.1μg.kg-1.h-1和5μg.kg-1.h-1维持;右美托咪定组输注右美托咪定1μg.kg-1.h-1和芬太尼10μg.kg-1.h-1,1 h后分别以0.5μg.kg-1.h-1和5μg.kg-1.h-1维持。必要时以0.4%～1.0%异氟醚吸入维持麻醉。监测并记录记录麻醉诱导前、麻醉后1 h、切皮前、切皮后即刻、手术结束即刻、手术结束后10 min的血压和心率。结果两组患儿在输注麻醉药物1 h后,收缩压和心率均显著降低,差异有统计学意义(均P<0.05);在切皮时,咪达唑仑组收缩压、舒张压和心率较切皮前明显增高,且明显高于右美托咪定组,差异有统计学意义(均P<0.05);右美托咪定组较少患儿需加用异氟醚,与咪达唑仑组比较,差异有统计学意义[35.3%(12/34)vs.85.3%(29/34),χ2=17.752,P=0.000]。结论与咪达唑仑比较,右美托咪定可更有效的维持小儿先天性心脏病外科手术麻醉过程中的血流动力学稳定。     

Postoperative arrhythmias in adults with congenital heart disease: Incidence and risk factors

Background

Little is known about the clinical impact of arrhythmias after surgery for congenital heart disease (CHD) in adults. Therefore, we investigated the prevalence of in-hospital arrhythmias after CHD surgery and their impact on clinical outcome.

Methods

This was a multicenter retrospective study and included adults who underwent congenital cardiac surgery between January 2009 and December 2011. Clinical events were defined as all cause mortality, heart failure (HF) requiring medical treatment, thrombo-embolic event, major infections and permanent pacemaker (PM) implantation.

Results

Overall, 419 patients were included (mean age 38 ± 14 years, 55% male). Arrhythmias occurred in 134 patients (32%) and included supraventricular tachycardia (SVT, n = 100), bradycardias (n = 47) and ventricular tachycardia (VT, n = 19). In multivariate analysis age ≥ 40 years at surgery (OR 2.48, 95% Cl 1.40–4.60, P = 0.003), NYHA class ≥ II (OR 2.42, 95% Cl 1.18–4.67, P = 0.009), significant subpulmonary AV-valve regurgitation (OR 2.84, 95% Cl 1.19–6.72, P = 0.018), coronary bypass time (OR 1.35/60 minute increase, 95% Cl 1.06–1.82, P = 0.019) and CK-MB (OR 1.05 per 10 U/L increase, 95% Cl 1.01–1.09, P = 0.021) were associated with in-hospital arrhythmias. Overall, 58 clinical events occurred in 55 patients (13%) and included in the majority of the cases permanent PM implantation (5%), HF (4%) and death (2%). In-hospital arrhythmias were independently associated with clinical events (OR 7.80, 95% CI 2.41–25.54, P = 0.001).

Conclusion

Arrhythmias are highly prevalent after congenital heart surgery in adults and are associated with worse clinical outcome. Older and symptomatic patients with significant valvular heart disease at baseline are at risk of in-hospital arrhythmias.     

Pulmonary vascular disease in adults with congenital heart disease

Pulmonary arterial hypertension of variable degree is commonly associated with adult congenital heart disease. Depending on size and location of the underlying cardiac defect as well as on repair status, pulmonary arterial hypertension may present with or without reversed shunting and associated cyanosis (ie, Eisenmenger syndrome). We review available data on etiology, clinical presentation, prognosis, and management strategies of pulmonary arterial hypertension in adult patients with congenital heart disease. In addition, we discuss the numerous complications associated with Eisenmenger syndrome, representing a multisystem disorder. Finally, we present general management strategies and emerging disease-targeting therapies.