Congenital Left Ventricular Splint in an Adult Patient with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

A 24‐year‐old woman presented with a recent increase in dyspnea on exertion and development of presyncope. The patient stated that she has reproducible episodes of dizziness and near fainting when she climbs a flight of stairs and activity is limited to a slow gait.     

Peripheral Pulmonary Artery Stenosis: An Unusual Case and Discussion of Genetic Associations

Peripheral pulmonary artery stenosis is a common congenital heart lesion associated with several genetic syndromes. We have reviewed the genetics of the lesion and present an unusual case of peripheral pulmonary stenosis involving a newly reported genetic deletion on chromosome 16. Further studies will be needed to confirm association of this genotype and phenotype.     

Respiratory Syncytial Viral Infection in an Infant with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly in children that requires necessary and urgent repair. We report a child who was hospitalized with respiratory failure due respiratory syncytial viral (RSV) infection and was subsequently diagnosed with ALCAPA. Aggressive treatment for RSV included synagis and nebulized ribavirin prior to surgical repair. After waiting 4 weeks for the RSV infection to resolve, she underwent successful left coronary artery reimplantation on hospital day 27 and has regained normal left ventricular size and function.     

Right Pulmonary Artery Obstruction Is a Long‐term Complication of Aortopulmonary Window Repair

The early outcomes of transaortic patch repair closure for aortopulmonary window are satisfactory, but the lifelong fate of the aorta and pulmonary artery remains unknown. We describe a 40‐year‐old patient with right pulmonary artery occlusion accompanied by aneurysmal dilation of the ascending aorta 38 years after transaortic repair of an aortopulmonary window. Operative findings revealed patch shrinkage and thrombotic occlusion of the right pulmonary artery. The dilated ascending aorta firmly adhered to the right pulmonary artery. After pulmonary artery thrombectomy, the right pulmonary artery was reconstructed and the dilated ascending aorta was replaced.     

Aortopulmonary Window in Adults: Diagnosis and Treatment of Late‐presenting Patients

Objectives. Aortopulmonary window is an uncommon condition, particularly so in adulthood because it is usually fatal in infancy or childhood if untreated. Very few cases of those who have survived to adulthood and been operated on successfully have been described. Our study aimed to provide clinical, investigative, surgical, and outcome details of such patients. Design and Setting. Retrospective study of consecutive adult patients with aortopulmonary window treated at a tertiary charitable cardiovascular institute in South India between 1996 and 2006. Results. Six adult patients successfully underwent aortopulmonary window closure. Five of the six patients had been correctly diagnosed on echocardiography, while one was only diagnosed after cardiac catheterization for unexplained pulmonary arterial hypertension. Four of the patients had large defects with severe pulmonary arterial hypertension, with pulmonary vascular resistance index (PVRI) ranging from 5.2 to 15.9 at baseline. All showed significant reversibility with oxygen administration, with PVRI on oxygen falling to between 0.6 and 2.2. These patients successfully underwent cardiopulmonary bypass. The other two patients with small lesions underwent ligation off‐pump. There was no early or late mortality among these patients. All were in New York Heart Association class I, on follow‐up ranging from 3 months to 8 years. Conclusions. Aortopulmonary window may rarely present in adulthood. The diagnosis can usually be made by careful echocardiography alone. Even in the presence of severe pulmonary arterial hypertension, if a significant reversibility in pulmonary vascular resistance can be demonstrated with oxygen, the condition can be successfully corrected with good long‐term outcomes.     

Urinary Interleukin‐18 and Urinary Neutrophil Gelatinase‐associated Lipocalin Predict Acute Kidney Injury Following Pulmonary Valve Replacement Prior to Serum Creatinine

Background. It is becoming increasingly recognized that manifestations of congenital heart disease (CHD) extend beyond the cardiovascular system. The factors contributing to renal dysfunction in patients with CHD are multifactorial, with acute kidney injury (AKI) at time of cardiac surgery playing a major role. AKI is often diagnosed based on changes in serum creatinine and estimated glomerular filtration rate (eGFR). Such measurements are often late and imprecise. Recent data indicate that urinary biomarkers interleukin‐18 (IL‐18) and neutrophil gelatinase‐associated lipocalin (NGAL) are earlier markers of AKI. We sought to determine the efficacy of urinary IL‐18 and NGAL for detecting early AKI in patients undergoing surgical pulmonary valve replacement (PVR). Methods. Twenty patients presenting for surgical PVR with a history of previous repair of a conotruncal anomaly were enrolled. Preoperative clinical data were measured and urine samples and serum creatinine were collected at 6, 12, 24, and 72 hours post bypass. Urine was evaluated for NGAL and IL‐18. AKI was determined using the Risk, Injury, Failure, Loss and End Stage Renal Disease (RIFLE) classification system. Results. Using the RIFLE classification system, seven patients (35%) were found to have AKI defined as a drop in the eGFR or an increase in serum creatinine. All seven patients with AKI had marked increase from preoperative baseline in urine IL‐18 (sixfold) and NGAL (26‐fold). Using NGAL and IL‐18, AKI was detected at 6 hours postoperatively, resulting in AKI being identified 12–36 hours prior to detection by conventional methods. No preoperative predictors for AKI were identified. Conclusion. Both NGAL and IL‐18 are early predictive biomarkers of AKI, and both increase in tandem after surgical PVR. Importantly, both rise before an increase in creatinine or a decrease in eGFR is present. Monitoring both biomarkers may allow for earlier detection and subsequent interventions to prevent AKI at time of surgery for CHD.     

Percutaneous Closure of a Large PDA in a 35‐Year‐Old Man with Elevated Pulmonary Vascular Resistance

The presence of a large patent ductus arteriosus (PDA) may result in significant pulmonary hypertension, which may not be reversible. We present the case of a 35‐year‐old man with pulmonary hypertension who had successful percutaneous closure of a large PDA with an Amplatzer muscular ventricular septal defect occluder and resolution of his pulmonary hypertension. The use of prior balloon test occlusion of the PDA suggested that the procedure would be successful, despite the lack of an immediate fall in the pulmonary artery pressure.     

No Inflammatory Response Related to Pulmonary Hemodynamics in Children with Systemic to Pulmonary Shunts

Objective. The hypothesis was that the levels of circulating inflammatory mediators are related to the degree of volume and pressure stress on the pulmonary vasculature in children with congenital systemic to pulmonary shunts. Design. Prospective, cross‐sectional study. Setting. Tertiary center covering all pediatric heart surgery and interventions in Norway. Patients. Seventy‐four children, aged 0–12 years, admitted for surgical or interventional treatment of congenital systemic to pulmonary shunts. Outcome Measures. Plasma levels of eight mediators of vascular inflammation and endothelial activation, sampled from different vascular compartments. Results. Patients with the most pronounced pulmonary flow and pressure stress demonstrated no elevation of inflammatory mediator levels when compared with healthy controls. No pulmonary production or uptake of the measured markers was found. Hemodynamic explanatory factors showed weak correlations to the inflammatory marker levels by univariate analysis. Age was the only factor that significantly explained inflammatory response in the multivariate model. The presence of Down syndrome, irrespective of hemodynamic category, was associated with elevated plasma levels of soluble tumor necrosis factor receptor I, when controlling for age. Conclusions. Inflammatory mediators show no significant relationship to pulmonary hemodynamics in children with systemic to pulmonary shunts. Children with Down syndrome may have an increased inflammatory response.     

Imaging of Extralobar Pulmonary Sequestration in a Patient with Tetralogy of Fallot with Pulmonary Atresia,Ventricular Septal Defect and Multiple Aorto‐pulmonary Collateral Arteries with Multidetector Computed Tomography

Complex pulmonary vascular blood supply is common in patients with tetralogy of Fallot with pulmonary atresia, major systemic to pulmonary collateral arteries and hypoplastic or deficient central pulmonary arteries. An extralobar lung sequestration, which has not been described previously in these patients, was imaged in a 6‐week‐old infant with multidetector computed tomography with sub‐millimeter resolution. Arterial and venous vessels were analyzed using three‐dimensional vascular exploration tools and results were confirmed with cardiac catheterization.     

Patients with Pulmonary Hypertension Related to Congenital Systemic‐to‐Pulmonary Shunts are Characterized by Inflammation Involving Endothelial Cell Activation and Platelet‐mediated Inflammation

Objective. We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts and the change in these markers during treatment with bosentan. Background. Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts is largely unknown. Patients and Methods. Plasma levels of several inflammatory mediators were determined by enzyme immunoassays in 14 children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts before and after 12 months treatment with bosentan, and compared with levels in 54 healthy controls. Results. The patients were characterized by increased plasma levels of von Willebrand factor (～2.5‐fold), C‐reactive protein (～3.5‐fold), and soluble CD40 ligand (～2.5‐fold) as compared with controls, representing markers of endothelial cell activation, systemic inflammation, and platelet‐mediated inflammation, respectively. Patients also had significantly elevated plasma levels of osteoprotegerin (～1.6‐fold). Within the study group, N‐terminal pro‐brain natriuretic peptide levels correlated significantly with the concentrations of C‐reactive protein (r= 0.61, P < .027) and von Willebrand factor (r= 0.74, P= .004). Except for a decline in monocyte chemoattractant protein‐1 and receptor activator of nuclear factor‐κB ligand, bosentan therapy did not attenuate the systemic inflammation. Conclusion. Children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts are characterized by enhanced systemic inflammation involving increased endothelial cell activation and platelet‐mediated inflammation. These inflammatory responses seem essentially to be unmodified by bosentan, potentially representing new targets for therapy in this disorder.     

Outcome of Pulmonary Valve Replacements in Adults after Tetralogy Repair: A Multi‐institutional Study

Objective. The purpose of this study was to assess the outcome of pulmonary valve replacement (PVR) in adults with moderate/severe pulmonary regurgitation after tetralogy repair, with particular emphasis on patient outcome, durability of valve repair, and improvement in symptomatology. Design/Setting/Patients. The project committee of the International Society of Congenital Heart Disease undertook a retrospective multi‐institutional analysis of PVR. Seven centers participated in submitting data on 93 patients >18 years of age who had the operation performed and follow‐up obtained. The average age of PVR was 26± years (median 27 years). Time of follow‐up after replacement was 3 years (range 4 days–28 years). Outcomes/Measures/Results. Kaplan–Meier estimates of durability of PVR showed approximately 50% replacement at 11 years. There were two deaths at 6 and 12 months after valve replacement. Right ventricular (RV) size estimated by echocardiography from pre‐ to postoperative studies decreased in 81% (P < 0.001 testing for equal proportions), but RV systolic function increased in only 36% (P = 0.09). Ability index improved in 59% (P < 0.001) and clinical heart failure status improved in 57% with this problem before PVR. PVR did not improve arrhythmia status in a small group of patients. Conclusions. PVR is associated with low mortality, decrease in RV size and improvement in ability index, and uncertain effects on RV systolic function. Average valve durability was approximately 11 years. Criteria for PVR that will preserve RV function are not clearly identified, and management of these patients remains a difficult enterprise.     

Pulmonary Vein Isolation for the Treatment of Drug‐Refractory Atrial Fibrillation in Adults with Congenital Heart Disease

Background. Atrial fibrillation (AF) is a common arrhythmia in adults with congenital heart disease (CHD). Long‐term antiarrhythmic therapy (AAT) in these patients has significant shortcomings. The safety and efficacy of pulmonary vein antrum isolation (PVAI) for the treatment of AF in CHD is presently unknown. Hypothesis. We hypothesized that PVAI for AF in patients with CHD is effective and safe. Methods. We reviewed a prospective cohort of 4315 patients (age ≥ 18) undergoing PVAI for drug refractory AF at a single institution and identified 36 consecutive patients with CHD (single ventricle physiology, tetralogy of Fallot, coarctation of the aorta, ventricular septal defects, atrial septal defects (ASD) and cardiomyopathy resulting from anomalous origin of the left main coronary from the pulmonary artery). A second cohort of 355 consecutive patients with noncongenital structural heart disease (NSHD) (coronary artery disease, valvular heart disease, ejection fraction <50%, or prior noncongenital cardiac surgery) undergoing PVAI during the same time period was used as a control. Success was defined as freedom from AF starting two months after PVAI in the absence AAT until the end of follow‐up. Partial success was defined as freedom from AF in the presence of AAT until the end of follow‐up. Combined success was defined as the sum of success and partial success. We compared the outcomes with the use of propensity‐score matching in the overall cohort. Results. Patients with NSHD were older and had higher prevalence of hypertension (P < .01), diabetes (P < .01) and hyperlipidemia (P < .01). The most common CHD lesion was ASD (61%) and the most common NSHD lesion was valvular heart disease (57%). After one PVAI, success was achieved in 42% and 53% at 300 days in the CHD and NSHD groups respectively. Four‐year success was achieved in 27% and 36% in the CHD and NSHD groups, respectively. There were no significant differences in the success rates between patients groups (P= .46), nor were there any differences in left atrial size or changes in ejection fraction after one or two PVAI in the respective groups. Complication rates between the CHD and NSHD groups were similar (15% vs. 11%, P= .42) except for a higher risk of vascular site complications in patients with CHD (8% vs. 1%, P < .05). Conclusion. PVAI is an attractive treatment modality in drug refractory AF in CHD, with combined success rates in excess of 60%. The maintenance of sinus rhythm after PVAI in CHD appears similar to that of NSHD and warrants prospective validation.     

Permanent Transvenous Left Ventricular Endocardial Pacing in a Patient with Univentricular Atrioventricular Connection

Chronotropic incompetence is associated with poorer effort tolerance and worse mortality outcomes, not only in the general cardiac population but also in patients with congenital heart disease. When present in complex patients, pacing options may be limited by difficult pacing access, and an open surgical approach for epicardial lead placement may not always be desirable. We describe a case of symptomatic chronotropic incompetence in a patient with tricuspid atresia, valvar and subpulmonary stenosis, normally related great vessels, and a modified Blalock–Taussig shunt, awaiting cardiac transplantation, whom we palliated with a transvenous endocardial pacing strategy. This technique may provide an alternative pacing strategy for highly selected patients, where few other treatment options are available.     

Fetal Goiter Mimicking Anomalous Pulmonary Venous Connection

Fetal thyroid dysfunction is a well-recognized cause of secondary cardiac disease, including arrhythmias and hydrops fetalis, but has not previously been reported to mimic structural heart disease. We describe a case of fetal goiter presenting as suspected anomalous pulmonary venous connection and highlight lessons for the obstetrician and pediatric cardiologist with regard to imaging as well as communication.