Four successful pregnancies in a patient with Fontan palliation and congenital heart disease: a case report.

BACKGROUND: Fontan is a palliative procedure in patients with single ventricle. Single ventricle supports systemic cardiac output and pulmonary blood flow is passively directed to the right pulmonary artery. Women with Fontan palliation are reported to have increased maternal risk during pregnancy. There are few reports of successful pregnancies in such cases. However data on these pregnancies is lacking, we consider this to be the first reported from kingdom of Saudi Arabia. CASE PRESENTATION: We present a 35-year-old woman from the Kingdom of Saudi Arabia who had Fontan surgery and who had four successful pregnancies and multiple miscarriages. She delivered live, low birth weight neonates. CONCLUSION: This report provides an anecdotal evidence that pregnancy can be tolerated in an adequate Fontan patient with successful outcomes.

Systemic arteriovenous fistulae for end-stage cyanosis after cavopulmonary connection: a useful bridge to transplantation.

OBJECTIVE: Intractable cyanosis after partial or complete cavopulmonary connection may rarely be managed by creating a systemic arteriovenous fistula. We investigated the long-term performance of arteriovenous fistulae. METHODS: All 21 patients who received an arteriovenous fistula at The Hospital for Sick Children since the 1950s were investigated using parametric competing risk techniques. Primary arteriovenous fistula indication was (1) suboptimal pulmonary blood flow (N = 15) or (2) pulmonary shunting via pulmonary arteriovenous malformations (N = 6). Arteriovenous fistula longevity was determined by time to "occlusion" (absence of arteriovenous fistula flow via surgical ligation or spontaneous occlusion). RESULTS: All 21 patients had previously undergone second-stage palliation (Glenn shunt = 13; bidirectional shunt = 9). Five patients had undergone Fontan completion. Death in the presence of a functioning arteriovenous fistula occurred in 5 patients. Patients with bidirectional shunts had a significantly higher risk of death with a functioning arteriovenous fistula in situ (P = .04). High hemoglobin concentrations were associated with best outcome, and levels less than 170 g/L were associated with a high risk of death despite a functioning arteriovenous fistula (P < .01). Arteriovenous fistula occlusion occurred in 10 patients. Earlier occlusion was associated with previous Fontan completion (P = .02) and pulmonary arteriovenous malformations (P = .03). Surgical ligation during cardiac transplantation was the cause of occlusion in 7 patients. In these 7 patients, the arteriovenous fistula functioned for a median of 4.8 years. After transplantation, survival was 67% + or - 19% at 5 years. Overall survival was 73% + or - 10% 15 years after receiving an arteriovenous fistula (longest survival, 27.3 years). CONCLUSION: In patients with adequate hematocrit, arteriovenous fistula offers an effective bridge to transplantation when a high-risk Fontan procedure is deferred. Performance is best after unidirectional cavopulmonary connection and worse in the presence of pulmonary arteriovenous malformations. Survival is 75% at 15 years, despite being considered end stage.

Mid-term outcomes in adults with ebstein anomaly and cavopulmonary shunts.

BACKGROUND: In patients with Ebstein anomaly and poorly functioning right ventricles, a cavopulmonary shunt (CPS) can be created to reduce the preload on the right ventricle. The purpose of this study was to examine the early and mid-term outcomes in adults with Ebstein anomaly who have undergone tricuspid valve repair or replacement with or without a concomitant CPS. METHODS: We examined the outcomes of 40 consecutive patients seen at our center with Ebstein anomaly who had undergone tricuspid valve repair or replacement with (n = 23) or without (n = 17) concomitant CPS. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term survival was examined using Kaplan-Meier curves. RESULTS: The mean age at surgery was similar in patients with and without CPS (42 +/- 12 versus 39 +/- 19 years; p = 0.63). There were 2 early postoperative deaths owing to refractory right-sided heart failure. Mid-term follow-up data were available in 95% of patients. The mean follow-up time was 6.7 +/- 4.8 years. Patients who received a CPS more commonly had preoperative heart failure or cyanosis (p = 0.04) and had worse preoperative functional status (p = 0.09). In both groups, arrhythmias were the most common late complication. There were 5 late deaths, 3 of which occurred in patients with CPS. Five-year survival with or without CPS was comparable (83% +/- 9% versus 86% +/- 10%; p = 0.85). CONCLUSIONS: Adolescent and adult patients with Ebstein anomaly undergoing tricuspid valve replacement or repair and concomitant CPS are at risk for early and mid-term complications. However, Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not thought to be suitable for tricuspid valve surgery alone.