Contemporary Management of the Failing Fontan.

Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital heart disease seen in adult patients is the Fontan circulation. These patients have complex physiology and are at risk for several complications, including thrombosis of the Fontan pathway, pulmonary vascular disease, heart failure, atrial arrhythmias, atrioventricular valve regurgitation, and protein-losing enteropathy. This review discusses the commonly encountered phenotypes of Fontan circulatory failure and their contemporary management.

Predictors of arrhythmia during pregnancy in adults with congenital heart disease.

BACKGROUND: Risk prediction of arrhythmia during pregnancy in adult congenital heart disease (ACHD) patients is currently lacking, and the impact of preconception catheter ablation on future antepartum arrhythmia has not been studied. METHODS: We conducted a single-center, retrospective cohort study of pregnancies in ACHD patients. Clinically significant arrhythmia events during pregnancy were described, predictors of arrhythmia were analyzed, and a risk score devised. The impact of preconception catheter ablation on antepartum arrhythmia was assessed. RESULTS: The study included 172 pregnancies in 137 patients. Arrhythmia events occurred in 25 (15%) of pregnancies, with 64% of events occurring in the second trimester and sustained supraventricular tachycardia being the most common rhythm. Univariate predictors of arrhythmia were history of tachyarrhythmia (OR 20.33, 95% CI 6.95-59.47, p < 0.001, Fontan circulation (OR 11.90, 95% CI 2.60-53.70, p < 0.001), baseline physiologic class C/D (OR 3.72, 95% CI 1.54-9.01, p = 0.002) and history of multiple valve interventions (OR 3.10, 95% CI 1.20-8.20, p = 0.017). Three risk factors (excluding multiple valve interventions) were used to formulate a risk score, with a cutoff of ≥2 points predicting antepartum arrhythmia with sensitivity and specificity of 84%. While recurrence of the index arrhythmia was not observed following successful catheter ablation, preconception ablation did not impact odds of antepartum arrhythmia. CONCLUSIONS: We provide a novel risk stratification scheme for predicting antepartum arrhythmia in ACHD patients. The role of contemporary preconception catheter ablation in risk reduction needs further refinement with multicenter investigation.

Outcomes of malignancy in adults with congenital heart disease: a single center experience.

BACKGROUND: Malignancy is known to be a major cause of death in adult congenital heart disease (ACHD). However, data regarding cardiovascular and cancer-related outcomes in ACHD are lacking. METHODS: We conducted a retrospective single-center cohort study comprising patients with ACHD and malignancy. The primary outcome was all-cause mortality. Key secondary outcomes included major adverse cardiovascular and cerebrovascular events (MACCE), cardiotoxicity events and consequent cancer therapy modifications. RESULTS: Sixty-eight patients with ACHD and a history of cancer were included in the study. 82% of patients had moderate or great ACHD anatomic complexity. Over a median follow-up of 5 years after cancer diagnosis, 16 (24%) patients died, with 69% of deaths being due to cancer. Univariate predictors of mortality were baseline arrhythmia (OR 3.82, 95% CI 1.15-12.67, p = 0.028), baseline diuretic therapy (OR 3.54, 95% CI 1.04-12.04, p = 0.044) and advanced cancer stage at diagnosis (OR 2.37, 95% CI 1.32-4.25, p = 0.004). MACCE occurred in 40 (59%) patients and was independently predicted by baseline diuretic requirement (OR 9.91, 95% CI 1.12-87.85, p = 0.039). A 14% incidence of cardiotoxicity was seen; 3 patients needed modification and 1 patient needed temporary interruption of cancer therapy for 2 weeks. CONCLUSIONS: Considerable mortality occurred in this cohort of patients with ACHD and cancer; most deaths were cancer-related. A high rate of MACCE was observed, yet rates of obligatory modification of cancer therapy due to cardiotoxicity were low.

Right coronary artery originating from the left ventricular outflow tract diagnosed after a Ross procedure: a case report.

Background: Anomalous coronary origin from the left ventricular outflow tract (LVOT) is an exceedingly rare condition thought to be associated with the bicuspid aortic valve (BAV). While the malignant presentation of this entity has been described, its pathophysiology and diagnostic evaluation are poorly understood. Case summary: A 33-year-old woman status post Ross procedure in childhood for congenital aortic stenosis due to BAV with presumed common origin of right and left coronary arteries based on single coronary ostium seen on aortic valve inspection, presented with symptomatic pulmonary regurgitation and stenosis. Invasive left coronary angiography revealed retrograde filling of the right coronary artery (RCA) with systolic washout of contrast indicating a patent RCA ostium. No RCA ostium was found on aortic root injection, but an injection into the LVOT revealed an RCA ostium below the aortic valve. Selective RCA angiography revealed pulsatile antegrade flow down the RCA occurring during systole. There was no anatomic RCA stenosis. We proceeded with valve-in-valve TcPVR. The patient had significant improvement of symptoms and RCA reimplantation was hence deferred. Discussion: This case is the first of an anomalous coronary artery arising from the LVOT diagnosed in a patient after the Ross procedure. Our angiograms shed light on the unusual physiology of coronary filling during systole and ischaemia arising from inadequate perfusion gradient between the left ventricle and the coronary during systole, leading to collateralization despite the lack of anatomic stenosis. We urge consideration of this potentially malignant entity in any symptomatic patient, especially with concomitant BAV.

Aortic Rupture as a Complication of Cardiopulmonary Resuscitation.

A 78-year-old woman with an aortic pseudoaneurysm suffered cardiac arrest due to aspiration and underwent cardiopulmonary resuscitation (CPR), but was found to then be hypotensive with a new chest hematoma. Imaging revealed aortic rupture at the site of the pseudoaneurysm. Aortic rupture is a rare but catastrophic complication of CPR. (Level of Difficulty: Beginner.).

Predictors of Late Mortality in D-Transposition of the Great Arteries After Atrial Switch Repair: Systematic Review and Meta-Analysis.

Background Existing data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D-transposition of the great arteries (D-TGA) are heterogeneous and limited by statistical power. Methods and Results We conducted a systematic review and meta-analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow-up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter-defibrillator therapy in this population. Average survival dropped to 65% at 40 years after atrial switch repair, with sudden cardiac death accounting for 45% of all reported deaths. Mortality was significantly lower in cohorts that were more recent and operated on younger patients. Patient-level risk factors for late mortality were history of supraventricular tachycardia (odds ratio [OR] 3.8, 95% CI 1.4-10.7), Mustard procedure compared with Senning (OR 2.9, 95% CI 1.9-4.5) and complex D-TGA compared with simple D-TGA (OR 4.4, 95% CI 2.2-8.8). Significant risk factors for sudden cardiac death were history of supraventricular tachycardia (OR 4.7, 95% CI 2.2-9.8), Mustard procedure (OR 2.2, 95% CI 1.1-4.1), and complex D-TGA (OR 5.7, 95% CI 1.8-18.0). Out of a total 124 implantable cardioverter-defibrillator discharges over 330 patient-years in patients with implantable cardioverter-defibrillators for primary prevention, only 8% were appropriate. Conclusions Patient-level risk of both mortality and sudden cardiac death after atrial switch repair are significantly increased by history of supraventricular tachycardia, Mustard procedure, and complex D-TGA. This knowledge may help refine current selection practices for primary prevention implantable cardioverter-defibrillator implantation, given disproportionately high rates of inappropriate discharges.

Five-year mortality outcomes in patients with chronic kidney disease undergoing percutaneous coronary intervention.

OBJECTIVE: To examine peri-procedural and long-term outcomes in patients with chronic kidney disease (CKD) undergoing percutaneous coronary interventions (PCI). BACKGROUND: Patients with advanced CKD are considered high risk when undergoing PCI. Limited published data exist on quantifying risk and assessment of long-term outcomes after PCI in this group. METHODS: Examining the Cornell Coronary Registry, we prospectively collected data of 6,478 consecutive patients who underwent elective or urgent PCI between 2009 and 2013. Patients were grouped into CKD stages by estimated glomerular filtration rate (eGFR) according to KDOQI guidelines. Procedural and 30-day outcomes are reported with assessment of long-term differences in 5-year all-cause mortality. RESULTS: Patients were grouped by CKD stages: 1,351 patients with eGFR ≥90 mL/min/1.73 m2 (stage 1), 2,882 with eGFR 60-89 (stage 2), 1,742 with eGFR 30-59 (stage 3), 191 with eGFR 15-29 (stage 4), and 312 with eGFR <15 or on dialysis (stage 5). The incidence of post-procedural acute heart failure, stroke, new dialysis requirement, transfusions, and bleeding events were higher in patients with greater CKD stage (P < 0.05). Five-year Kaplan-Meier overall survival among CKD stages 1-5 was 98.1, 95.5, 91.8, 82.5, and 76.9%, respectively (P < 0.001 by log-rank test). The hazard ratios of all-cause mortality for CKD stages 2-5 as compared to stage 1 by multivariate Cox regression analysis were as follows: 1.32 (P = 0.26), 2.04 (P < 0.01), 2.79 (P < 0.01), and 5.49 (P < 0.001). CONCLUSION: Among patients undergoing PCI, lower GFR is associated with decreased long-term survival. © 2016 Wiley Periodicals, Inc.

Portal, superior mesenteric and splenic vein thrombosis secondary to hyperhomocysteinemia with pernicious anemia: a case report.

INTRODUCTION: Acute portomesenteric vein thrombosis is an uncommon but serious condition with potential sequelae, such as small-bowel gangrene and end-stage hepatic failure. It is known to be caused by various pro-thrombotic states, including hyperhomocysteinemia. We describe what is, to the best of our knowledge, the first reported case of concomitant thrombosis of portal, superior mesenteric and splenic veins due to hyperhomocysteinemia secondary to pernicious anemia and no other risk factors. CASE PRESENTATION: A 60-year-old Indian man presented with epigastric pain, diarrhea and vomiting. An abdominal imaging scan showed that he had concomitant pernicious anemia and concomitant portal, superior mesenteric and splenic vein thrombosis. A work-up for the patient's hypercoagulable state revealed hyperhomocysteinemia, an undetectable vitamin B12 level and pernicious anemia with no other thrombophilic state. He developed infarction with perforation of the small bowel and subsequent septic shock with multi-organ dysfunction syndrome, and he ultimately died due to progressive hepatic failure. CONCLUSION: This report demonstrates that pernicious anemia, on its own, can lead to hyperhomocysteinemia significant enough to lead to lethal multiple splanchnic vein thrombosis. Our case also underscores the need to (1) consider portomesenteric thrombosis in the differential diagnosis of epigastric abdominal pain, (2) perform a complete thrombotic work-up to elucidate metabolic abnormalities that could be contributing to a pro-thrombotic state and (3) initiate aggressive measures, including early consideration of multi-visceral transplantation, in order to avoid decompensation and a significant adverse outcome.