To Total Cavopulmonary Connection Research Articles

Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavopulmonary Connection on Pulmonary Artery Growth.

Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC). A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs. Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, P < .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m2/year, 95% CI: 5.6-11.2, P < .001; 7.9 mm/m2/year, 95% CI: 5.5-10.2, P < .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw (P = .71, .70) or BSA-adjusted measurements (P = .86, .64). Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.

The mid-term outcome of Fontan conversion compared with primary total cavopulmonary connection

Background: The indication of Fontan conversion (FC) from atriopulmonary connection (APC) to total cavopulmonary connection (TCPC) is unclear. We sought to analyze the mid-term outcome of prophylactic and therapeutic Fontan conversion compared with that of primary TCPC. Methods: Patients with a univentricular heart who underwent cardiac catheterization at >18 years of age between July 2005 and July 2019 were included and divided into three groups: symptomatic APC patients who underwent therapeutic FC (t-FC, n = 13), asymptomatic APC patients after prophylactic FC (p-FC, n = 15), and patients who had primary TCPC procedure (pTCPC, n = 24). Results: The mean last follow up was at the age of 32.0 ± 7.8, 26.8 ± 3.8, and 27.3 ± 7 years (p = 0.07) in t-FC, p-FC, and pTCPC, respectively. There was no late death. All of t-FC and 12 (80%) of p-FC cases underwent concomitant arrhythmic surgery. Consequently, five and four patients in t-FC and p-FC groups required pacemaker implantations mostly due to sinus node dysfunction. Thromboembolism was seen in 2 cases in both t-FC (15%) and p-FC (13%), and 1 case in pTCPC (4%) (p = 0.50). The last cardiac catheterization was performed at the age of 29.5 ± 8.5, 24.6 ± 3.8, and 26.3 ± 7.1 years (p = 0.11) in t-FC, p-FC, and pTCPC patients, respectively. There was no significant difference in central venous pressure, aortic pressure, and cardiac index among the three groups. There was no late supraventricular tachyarrhythmic event seen in t-FC and p-FC, whereas two patients in pTCPC had newly developed atrial flutter. Conclusions: FC is a safe and feasible procedure to bring APC patients back onto the same track of primary TCPC patients in terms of hemodynamics as well as arrhythmia. The antiarrhythmic procedure should be carefully chosen because sinus node dysfunction can frequently occur and FC itself would reduce the risk of arrhythmia.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation.

BackgroundThe Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment.ObjectiveTo evaluate the results of conversion from FK to TCPC.MethodsA retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05.ResultsFontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured.ConclusionsThe conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

Outcomes of Fontan conversion for failing Fontan circulation: mid-term results.

We investigated the results of a revision of a previous Fontan connection to total cavopulmonary connection (TCPC) in patients with failing Fontan circulation. From July 1998 to April 2013, 21 patients who had failing Fontan circulation underwent revision of the previous Fontan operation to TCPC. The median age at TCPC conversion was 17.9 years (range, 4.6-38.1 years) and the median interval between initial Fontan operation and TCPC was 13.8 years (range, 2.1-25.4 years). There were 37 indications for Fontan revision in 21 patients. The indications were huge right atrium (n = 15), atrial arrhythmia (n = 8), intra-atrial thrombi (n = 6), protein-losing enteropathy (PLE) (n = 3) and more than mild atrioventricular valve regurgitation (n = 5). The previous Fontan operation was revised to extracardiac conduit replacement (n = 20) and intra-atrial lateral tunnel (n = 1). Concomitant surgery for atrial arrhythmia was performed in 8 patients. Fenestration was performed in 7 patients. The median follow-up duration was 7.1 years (range, 0.3-13.4 years). There were no operative deaths and two late deaths occurred 7.9 and 8.1 years after operation. Actuarial 5- and 10-year survival rates were 92.3 and 83.1%, respectively. Postoperative complications included bleeding (n = 3), deep sternal infection (n = 1) and prolonged pleural effusion for more than 2 weeks (n = 5). During follow-up, atrial arrhythmia recurred in 6 patients, PLE recurred in 2 patients and pleural effusion recurred in 2 patients. All patients were classified as New York Heart Association Class I (n = 15) or Class II (n = 4). Fontan conversion to TCPC in patients with failing Fontan circulation can be performed with low risk of morbidity and mortality. The procedure confers better quality of life and is functional for patients with failed Fontan circulation.

Early and intermediate-term results of the extracardiac conduit total cavopulmonary connection for functional single-ventricle hearts.

Fontan operation has evolved from atriopulmonary connection to total cavopulmonary connection (TCPC) due to its advantages in terms of hemodynamics and reduction of atrium-related complications. We analyzed the early and intermediate-term results of extracardiac conduit TCPC (EC-TCPC) procedure in patients with functional single ventricle to investigate the risk factors of surgical mortality and intermediate failure. Retrospective review of the medical records of 88 consecutive patients with functional single ventricle who underwent EC-TCPC from 2000 to 2013 was conducted. The follow-up was 100% complete, ranging from 3months to 13years (mean 7.0±3.8years). There were two (2.3%) hospital and 18 (20.4%) late deaths. The estimated event-free survival rates at 1year, 5years, and 10years were 90.6%, 89.3%, and 77.2%, respectively. On univariate analysis, fenestration was the only risk factor for surgical mortality (p=0.027). On multivariate analysis, the significant atrioventricular valve regurgitation was the only risk factor for intermediate failure (p=0.017). The clinical results of EC-TCPC in patients with functional single ventricle were satisfactory. The patients who needed fenestration during operation had higher risk of surgical mortality. Significant atrioventricular valve regurgitation had negative impact on intermediate survival.

Modified Fontan conversion: an alternative technique for grown-up patients

A need persists for Fontan conversion (classical Fontan to total cavopulmonary connection (TCPC)) that provides alternative approaches for the individual anatomical challenges occurred in these unusual and complex congenital grown-up patients.

Drug-induced QT-interval prolongation and recurrent torsade de pointes in a child with heterotaxy syndrome and KCNE1 D85N polymorphism

We present a child case of heterotaxy syndrome (asplenia syndrome) after Fontan procedure that showed extreme prolongation of QT interval and torsade de pointes (TdP) after administration of sodium channel blockers for paroxysmal atrial tachycardia. Despite low serum concentration of the drugs, QT prolongation persisted and TdP attacks with unconsciousness recurred, possibly in association with junctional bradycardia and myocardial damage although he had never experienced QT prolongation during bradycardia before. Temporal cardiac pacing via a venous route to exclude possible implication of bradycardia in induction of TdP was difficult to apply due to total cavopulmonary connection (TCPC) circulation. Continuous intravenous administration of low-dose isoproterenol was started but an appropriate heart rate for prevention of TdP was difficult to obtain. Finally, we were urged to conduct implantation of a DDD pacemaker combined with ICD surgically with epicardial leads, resulting in successful suppression of TdP and syncope. Screening of the genotype disclosed the KCNE1 D85N polymorphism, which is known as one of the typical disease-causing gene variants in long-QT syndrome (LQTS).

Systemic to pulmonary collateral blood flow influences early outcomes following the total cavopulmonary connection

BackgroundSystemic to pulmonary collaterals (SPCs) represent an additional and unpredictable source of pulmonary blood flow in patients with single ventricle physiology following bidirectional superior cavopulmonary connection (BCPC). Understanding their influence...

Arrhythmia Recurrence in Adult Patients with Single Ventricle Physiology Following Surgical Fontan Conversion

To evaluate the incidence of atrial tachy-arrhythmia (AT) recurrence following conversion from right atrial-pulmonary artery (RA-PA) Fontan to total cavopulmonary connection (TCPC) in adults. AT is a recognized sequel of Fontan palliation, especially in RA-PA Fontans, and is associated with significant morbidity. While catheter ablation achieves fairly reliable short-term success with low morbidity, conversion to TCPC with arrhythmia surgery is a highly effective treatment option for the classical Fontan patients with incessant AT. Single center retrospective review. Twenty-seven adults underwent Fontan conversion from RA-PA to TCPC, mostly for AT indications (n = 24). Nine (33%) underwent conversion to a lateral tunnel (LT) and 18 (67%) to an extracardiac (EC) Fontan. Two patients died <30 days post-operatively. Both had liver failure and had been turned down for cardiac/liver transplantation. In-hospital complications occurred in 15/27 patients (55%), including recurrence of AT requiring cardioversion in six patients (22%) and persistent pleural effusions in 4 (15%). Mean follow-up was 4.2 years (range 3 months-14 years). Functional capacity improved from mean New York Heart Association (NYHA) class 1.8 pre-conversion to 1.2 post-conversion (P= 0.008). Twenty-one patients had concomitant arrhythmia surgery (MAZE in 12 patients with IART and Cox-MAZE in nine patients with A-Fib +/- IART). Of these, 3/21 (14%) had AT recurrence >3 months following conversion. Conversion from RA-PA Fontan to TCPC, with arrhythmia surgery, decreases AT recurrence and improves functional capacity. The risk of peri-operative mortality is highest in patients with cirrhosis. AT recurred in 14% of patients.

ARRHYTHMIA RECURRENCE IN ADULT PATIENTS WITH SINGLE VENTRICLE PHYSIOLOGY FOLLOWING SURGICAL FONTAN CONVERSION

To evaluate the incidence of atrial tachy-arrhythmia (AT) recurrence following conversion from right atrial-pulmonary artery (RA-PA) Fontan to total cavopulmonary connection (TCPC) in adults.AT is a recognized sequel of Fontan palliation, especially in RA-PA Fontans, and is associated with significant morbidity. While catheter ablation achieves fairly reliable short-term success with low morbidity, conversion to TCPC with arrhythmia surgery is a highly effective treatment option for the classical Fontan patients with incessant AT.Single center retrospective review.Twenty-seven adults underwent Fontan conversion from RA-PA to TCPC, mostly for AT indications (n = 24). Nine (33%) underwent conversion to a lateral tunnel (LT) and 18 (67%) to an extracardiac (EC) Fontan. Two patients died <30 days post-operatively. Both had liver failure and had been turned down for cardiac/liver transplantation. In-hospital complications occurred in 15/27 patients (55%), including recurrence of AT requiring cardioversion in six patients (22%) and persistent pleural effusions in 4 (15%). Mean follow-up was 4.2 years (range 3 months-14 years). Functional capacity improved from mean New York Heart Association (NYHA) class 1.8 pre-conversion to 1.2 post-conversion (P= 0.008). Twenty-one patients had concomitant arrhythmia surgery (MAZE in 12 patients with IART and Cox-MAZE in nine patients with A-Fib +/- IART). Of these, 3/21 (14%) had AT recurrence >3 months following conversion.Conversion from RA-PA Fontan to TCPC, with arrhythmia surgery, decreases AT recurrence and improves functional capacity. The risk of peri-operative mortality is highest in patients with cirrhosis. AT recurred in 14% of patients.

Management of late atrial tachyarrhythmia long after Fontan operation

The optimal management of atrial tachyarrhythmia (AT) late after Fontan operation has not yet been established. Of 199 patients who were followed for more than 10 years after Fontan operation, 60 patients in whom late postoperative arrhythmias were observed were the subjects of this study. These arrhythmias were managed with anti-arrhythmic drugs. Twenty-one of 60 patients (35%) did not respond to the drugs and they needed further interventions. Fourteen catheter ablation procedures were performed in nine patients (atrial fibrillation in one patient, AT in eight patients) and the success rate was 44%. Conversion to total cavopulmonary connection (TCPC) was performed in 14 patients and Maze operation was performed at the time of Fontan conversion in 6 patients. Sinus rhythm or pacemaker rhythm was maintained in 7 of 14 (50%) patients postoperatively. Although arrhythmogenic substrates accumulate and tachyarrhythmia becomes frequent over the long-term following Fontan operation, most patients with AT can be managed medically. The success rate of catheter ablation and Maze operation is low but those interventions may be indicated in patients with intractable arrhythmias.

Progressive intrapulmonary shunting in a child after bidirectional Glenn operation only resolved after total cavopulmonary completion

We report on a girl with progressive intrapulmonary shunting after bidirectional Glenn (BDG) operation and resolution of these microscopic fistulas after completion to total cavopulmonary connection (TCPC).

Effectiveness of Carvedilol for Congestive Heart Failure that Developed Long after Modified Fontan Operation

We report a case of a patient with severe heart failure after Fontan procedure in whom carvedilol was very effective. A 27-year-old man had intractable congestive heart failure due to severe ventricular dysfunction after Fontan operation. Central venous pressure was elevated to 29 mmHg. A right-to-left shunt was noted across a large collateral vessel between the innominate vein and the pulmonary vein. He was administered carvedilol (initial dose, 2 mg/day; maximum dose, 30 mg/day). Cardiac catheterization performed 1 year after carvedilol administration revealed a decrease in atrial pressure and improvement of ventricular function. He underwent a conversion operation to total cavopulmonary connection (TCPC) and ligation of a collateral vein communicating with the innominate and pulmonary veins. Carvedilol may be a legitimate treatment before TCPC conversion or heart transplantation for the high-risk group of patients with a failed Fontan circulation.

Systemic plasma vascular endothelial growth factor levels as a marker for increased angiogenesis during the single ventricle surgical pathway

Cyanosis and the cavopulmonary anastomosis (CPA) are associated with pulmonary arterio-venous malformations (PAVMs) in single ventricle physiology. Vascular endothelial growth factor (VEGF) may be a marker of abnormal angiogenesis in this setting. Plasma VEGF levels were measured in 14 patients undergoing the surgical pathway leading to total cavopulmonary connection (TCPC). Venous blood samples were taken before and then months after CPA (n=6), and immediately before TCPC and 1 month thereafter (n=9). Corresponding arterial saturations were correlated with VEGF levels at each time frame. In six patients, pre-CPA plasma VEGF levels rose from a mean of 24.4-112.4 pg/ml (p<0.03) just prior to completion of TCPC. In nine patients, VEGF levels diminished from 115.7 to 48.9 pg/ml (p<0.05) after TCPC. VEGF levels were disproportionately elevated to arterial saturations most notably after CPA (r2=0.002), suggesting an additional angiogenic stimulus besides cyanosis. Plasma VEGF levels fluctuate during the single ventricle surgical pathway, with maximal levels after CPA, and regression after completion of TCPC. High VEGF levels are disproportionate to hypoxia after CPA, potentially incriminating the absence of hepatic flow to the lungs as an abnormal angiogenic stimulus. Measuring VEGF in venous blood may serve as a biochemical marker of angiogenesis after CPA.

Total cavopulmonary connection for complex cardiac anomalies with the functional single ventricle.

From Aug. 1999 to Feb. 2001, 8 patients with complex congenital heart diseases, including 5 cases accompanied by hypoplastic left ventricle and 3 by hypoplastic right ventricle, were subjected to total cavopulmonary connection (TCPC). Eight cases underwent the operation under cardiopulmonary bypass and 7 of whom under no cardiac clamp. Seven cases received cavopulmonary anastomosis by flaring method and one case by end-side anastomosis. All the patients underwent the intracardiac tunnels to drain inferior vena cava and plus 4 mm fenestration except one. The results showed that 6 patients had postoperative oxygen saturation more than 90%, sinus rhythm, no anastomostic stoma obstruction, no flow regurgitation and CVP < 16 cmH2O. Two (25%) patients died postoperatively from high venous pressure of 18-20 cmH2O, finally from cardiac failure and anoxima. It was concluded that TCPC was an effective treatment for complex congenital cardiac diseases, especially with ventricular maldevelopment. Intracardiac tunnel plus 4 mm fenestration and flaring cavopulmonary anastomosis could prevent the postoperative complications. Larger anastomotic stoma, venous pressure less than 16 cmH2O and artery saturation more than 90% might indicate excellent TCPC procedures in our experience.