Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis†.

OBJECTIVES: This study aimed to investigate the indications for and the results of axillary arterio-venous fistula in patients with a functionally single ventricle at mid-term follow-up after bidirectional cavopulmonary connection. METHODS: From 2012 to 2017, 20 patients aged 21.1 ± 8.7 years (median 20.5) underwent axillary arterio-venous fistula after bidirectional cavopulmonary connection in our institution. The interval between bidirectional cavopulmonary connection and axillary arterio-venous fistula was 13.9 ± 7.2 years. The clinical and haemodynamic parameters of the patients were assessed before and after surgery. RESULTS: There was no in-hospital mortality. Paraesthesia of the arm persisted for 1-7 days. The mean arterial oxygen saturation increased from 72.4 ± 7.1% pre-surgery to 81.6 ± 5.5% (P < 0.001) after surgery, and was 83.8 ± 6.5% at discharge (P = 0.002 Wilcoxon matched pairs test). The haemoglobin, haematocrit, McGoon index and ejection fraction values did not change significantly. The speed and minute volume of the blood flow in the superior vena cava increased from from 21.1 ± 8.3 cm/s to 28.8 ± 14.9 cm/s (P < 0.001) and from 10.6 ± 3.7 ml/s to 12.5 ± 2.9 ml/s (P < 0.001), respectively. At the mid-term follow-up (1-5 years), 2 patients died from concomitant pathology. Stable improvement in the state and tolerance of physical activity was noted: before surgery, 17 patients were classified as New York Heart Association functional capacity (FC) III and 3 patients as FC IV; after surgery, 9 patients were FC II and 3 patients were FC III. One patient underwent a Fontan operation within 3 years. CONCLUSIONS: Axillary arterio-venous fistula can be used as the stage of surgical treatment and a bridge to Fontan and/or heart transplantation for this category of patients.

Repair of isolated partial anomalous pulmonary venous connection of the right upper pulmonary veins to the left vertical vein.

The most common form of partial anomalous pulmonary venous connection is drainage of the right pulmonary veins to the superior vena cava. On the contrary, partial anomalous venous connection of right pulmonary veins to the left vertical vein is described in few reports. The goal of this publication is to present the case of diagnosis and surgical repair of this extremely rare pathology in a 19-year old female. Methods of preoperative examination included but were not limited to spiral computed tomography and angiocardiography. Intraoperative examination confirmed that the collector of right upper pulmonary veins was connected to the vertical vein that continued to the innominate vein and then to the superior vena cava draining into the right atrium. During surgical intervention, the vertical vein was transected in the area of connection to the innominate vein with the suturing of inlet area. The collector of the right upper pulmonary veins was then anastomosed with the left atrial appendage. Postoperative course was uneventful. In conclusion, isolated partial anomalous pulmonary venous connection of the right upper pulmonary veins to the left vertical vein is a very rare congenital defect. Existing modern techniques contribute to the precise and timely diagnosis of this anomaly.

Results of Fontan operation in patients with atrioventricular valve regurgitation.

OBJECTIVES: The influence of concomitant atrioventricular valve regurgitation on the results of Fontan operation remains disputable. The goal of this study was to compare early and late results of Fontan operation performed in patients with mild or moderate-to-severe atrioventricular valve regurgitation. METHODS: For retrospective analysis, patients with atrioventricular valve insufficiency assessed by echocardiography were divided into three groups based on the severity of regurgitation and its repair or non-repair during Fontan operation: Group 1: unrepaired regurgitation 1-2+ (n = 33); Group 2: unrepaired regurgitation 3-4+ (n = 11); Group 3: repaired regurgitation 3-4+ (n = 35). Actuarial survival was estimated by the Kaplan-Meier method, followed by the log-rank test to compare survival curves between groups. RESULTS: Hospital mortality rates in Groups 1 through 3 reached 3, 27 and 14%, respectively (P = 0.015, Group 1 vs Group 2). The frequency of non-lethal complications did not significantly differ between groups. However, patients from Group 2 required significantly more intensive inotropic support, longer mechanical ventilation and had larger pleural effusion. Predicted survival after Fontan operation was the worst in Group 2 (P = 0.016, Group 2 vs Group 1). The frequency of non-lethal late complications was also the highest in Group 2 (50 vs 17 or 11%); however, the difference did not reach statistical significance (P = 0.13 and 0.069, respectively). The severity of atrioventricular valve regurgitation during the follow-up did not significantly change when compared with discharge after the repair in Group 2 or Group 3 (P = 0.19 and 0.52, respectively), and significantly increased in Group 1 (P = 0.003). However, this increase did not have clinical significance during the reported period of follow-up. CONCLUSIONS: If unrepaired, concomitant moderate-to-severe atrioventricular valve regurgitation significantly worsens the results of the Fontan procedure. Longer observation is needed to define the strategy in patients with mild atrioventricular valve regurgitation that remained unrepaired during Fontan operation.

Repair of congenital heart defects associated with single pulmonary artery.

BACKGROUND: Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. METHODS: Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n = 25) and ventricular septal defect (n = 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. RESULTS: Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm(2)·m(-2)). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 ± 0.11. CONCLUSIONS: Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects.

Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery.

OBJECTIVES: Experience with the palliative treatment of congenital heart defects (CHDs) associated with unilateral absence of the pulmonary artery (UAPA) is limited. There is a description of 32 interventions in the available literature. The aim of this retrospective study was to present our experience with palliative interventions in patients with cyanotic CHDs associated with UAPA and to suggest a rational surgical strategy. METHODS: Twenty-nine patients were subjected to palliative interventions. All of them had the following cyanotic CHDs with agenesis of the left pulmonary artery: tetralogy of Fallot (n = 26) or double outlet right ventricle (n = 3). Twenty patients were subjected to a single and 9 to multiple staged palliative operations. Patients were grouped according to the type of initial palliation to assess the clinical and haemodynamic results of each surgical intervention: Group 1: systemic-to-pulmonary shunts (n = 13); Group 2: transluminal balloon pulmonary valvuloplasty (n = 5); Group 3: palliative reconstruction of the right ventricular outflow tract (n = 11). The median age of patients at the initial palliative intervention was 2.6 years. Twenty-three of 27 discharged patients were followed up for a median period of 3 years. RESULTS: Hospital mortality in our series reached 7% (2 of 29 patients). Both lethal outcomes occurred after palliative reconstruction of the right ventricular outflow tract was performed as a sole intervention. The assessment of angiographic parameters has shown that palliative reconstruction of the right ventricular outflow tract provided more significant and uniform enlargement of the pulmonary artery than systemic-to-pulmonary shunts or transluminal balloon pulmonary valvuloplasty. Fifty-nine percent of patients (17 of 29 patients) were subjected to complete repair of CHDs during the follow-up. CONCLUSIONS: Palliative surgical treatment of CHDs associated with UAPA can be performed with a relatively low risk. Systemic-to-pulmonary artery shunt and transluminal balloon pulmonary valvuloplasty are methods of choice in patients with non-severe hypoplasia of the single pulmonary artery. The intravascular intervention is indicated more in patients with a prevailing valvular component of the pulmonary stenosis. Palliative reconstruction of the right ventricular outflow tract is a more favourable procedure for patients with a severe hypoplasia of the single pulmonary artery.

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy.

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.

Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults.

The purpose of this study is to analyze the immediate results of bidirectional cavopulmonary anastomosis (BCPA) and Fontan operations performed in adults, and to reveal the risk factors. During the years 1983-2010, 681 consecutive patients underwent BCPA or a Fontan operation. Fifty-three of 681 patients were more than 18 years of age. Twenty-nine adults underwent BCPA and 24 underwent a Fontan operation. Immediate results of surgical treatment were followed during the hospital period. The average number of exceeded 'operability' criteria by Choussat et al. [Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchhill Livingstone, 1977:559-566] was significantly higher in patients from the BCPA group compared to the Fontan group (1.3±0.8 vs. 0.9±0.7, P=0.034). Hospital mortality after BCPA in adults was 6.9% (2/29) and did not differ from children (7.1%, 19/268), P=0.634. Hospital mortality after Fontan operation in adults was 8.3% (2/24) and did not differ from children (11.9%, 43/360), P=0.419. The frequency of non-lethal hospital complications was higher in patients after a Fontan operation. Patients from the Fontan group more frequently developed arrhythmias and prolonged pleural effusions. Preoperative regurgitation at atrioventricular valves was an independent risk factor for hospital mortality and morbidity after a Fontan operation. BCPA and Fontan operations performed in adults are accompanied by good immediate results and considerably improves patients' condition.

Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery.

BACKGROUND: Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital heart defect that still represents a surgical challenge. The purpose of this study is to summarize our experience of surgical treatment of this complex lesion. METHODS: From 1983 to 2003, 27 patients with tetralogy of Fallot and unilateral absence of the left (n = 25) or right (n = 2) pulmonary artery underwent different surgical interventions. The age of patients ranged from 40 days to 37 years (median, 5.3 years). Pulmonary arterial Nakata index and Nakata index Z-score were used for the quantitative assessment of the contralateral pulmonary artery. Twenty patients underwent various palliative procedures, namely Blalock-Taussig or Gore-Tex shunt, transluminal balloon pulmonary valvuloplasty, and reconstruction of right ventricular outflow tract without ventricular septal defect closure. At a median interval of 3.6 years after palliation, 13 patients underwent complete repair of tetralogy of Fallot. In the other 7 patients, complete repair was performed as a primary intervention. RESULTS: Hospital mortality after palliation and after a complete repair was the same and reached 5%. Sixteen patients with the Nakata index greater than 200 mm2/m2 and Z-score greater than -4 survived after a complete repair. One of 4 patients with Nakata index less than 200 mm2/m2 and Z-score less than -4 died after surgery. CONCLUSIONS: Majority of patients with tetralogy of Fallot and unilateral absence of the pulmonary artery require palliative intervention as a first step of surgical treatment. Nakata index greater than 200 mm2/m2 and Nakata index Z-score greater than -4 are criteria for a successful complete repair.

Transcatheter coil embolization of coronary artery fistula.

Between December 1982 and August 2001, coil embolization of coronary artery-to-cardiac chamber fistula was attempted in 15 patients aged 11 months to 44 years (mean, 7.2 +/- 2.5 years). The fistulae connected the left anterior descending artery to the right ventricle in 4 patients, the right coronary artery to the right ventricle in 3, the right coronary artery to the right atrium in 3, the circumflex artery to the right ventricle in 2, the circumflex artery to the right atrium in 2, and the right coronary artery to the trunk of the pulmonary artery in 1. Complete fistula occlusion was achieved in 14 patients (93%); one had a residual shunt and underwent repeat embolization one year later, resulting in complete occlusion. There was one early death (7%) in a 4-year-old girl who developed femoral artery thrombosis and acute renal failure. Complications comprised migration of the coil into the pulmonary artery (2), femoral artery thrombosis (2), and perforation of the vessel wall by the guidewire (1) with immediate thrombosis and occlusion of the fistula (no coil was deployed). The 13 survivors with coils were followed up for 0.5 to 13 years; complete occlusion of the fistula was confirmed in all cases.