Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

BACKGROUND: The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. METHODS: Between March 2010 and December 2020, 58 patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. RESULTS: Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34 patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41 patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2 cases, the procedure was unsuccessful; the remaining 34 were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6 patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. CONCLUSIONS: Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure.

OBJECTIVES: Patients with hypoplastic left heart syndrome are at risk for neurodevelopmental impairment. Hypoxic-ischemic brain injury during neonatal treatment might be a relevant cause. We evaluated the association between cerebral oxygenation in the perioperative course of the Norwood procedure and neurodevelopmental outcome. METHODS: Cerebral tissue oxygen saturation (ScO2) was obtained by near-infrared spectroscopy for 24 hours before and 48 hours after surgery in 43 patients. Full-scale, verbal, and performance IQ scores were evaluated with the Wechsler Preschool and Primary Scale of Intelligence at a median of 4.5 years (range, 3.5-6.8 years). Cognitive functions were assessed with the German Kognitiver Entwicklungstest für das Kindergartenalter (KET-KID). RESULTS: Mean IQ scores and KET-KID percentile ranks were in the lower-normal range (full-scale IQ, 94 ± 11; verbal IQ, 97 ± 13; performance IQ, 93 ± 9; KET-KID global, 42 ± 27; verbal, 48 ± 29; nonverbal, 37 ± 23). Scores were below average (full scale IQ <85 or KET-KID <16th percentile) in 12 cases. Mean preoperative ScO2 was lower in patients scoring below average (56.8% ± 7.1% vs 61.7% ± 5.8%; P = .028) and was correlated with full-scale IQ (r = 0.495; P = .001), verbal IQ (r = 0.524; P = .001), and performance IQ (r = 0.386; P = .012) scores, and with global (r = 0.360; P = .018) and verbal (r = 0.395, P = .009) KET-KID scores. A relationship between IQ or KET-KID scores and postoperative ScO2 was not found. Gestational age, head circumference z-score, age at surgery, and postoperative length of stay were associated with IQ and KET-KID scores. CONCLUSIONS: Neurodevelopmental outcome was in the lower-normal range. Along with innate patient factors, preoperative cerebral tissue oxygenation is likely an important determinant of cognitive development.

Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation.

BACKGROUND: Assessment of systemic right ventricular (RV) function in patients with hypoplastic left heart syndrome is important during long-term follow-up after Fontan repair. Traditional echocardiographic parameters to evaluate systolic ventricular function are affected by loading conditions. The only generally accepted load-independent parameter of systolic function, end systolic elastance (Ees), requires invasive catheterization. Therefore, we sought to determine if parameters obtained by 2-dimensional speckle tracking (2DST) were affected by acute changes in preload and correlated with catheterization-derived indices of RV contractility in hypoplastic left heart syndrome patients after Fontan palliation. METHODS AND RESULTS: Fifty-two patients with hypoplastic left heart syndrome (median age, 6.6; range 2.9-22.2 years) were prospectively enrolled to have echocardiography and conductance catheter studies performed simultaneously. We compared traditional echo, 2-dimensional speckle tracking and catheterization-derived parameters during different states of preload at baseline and during dobutamine infusion. Global longitudinal strain (S) showed a tendency to decrease with preload reduction, whereas global longitudinal strain rate (SR) did not change (S: -17.7 ± 3.4% versus -16.9 ± 3.8%, P=0.08; SR: -1.30 ± 0.29 versus -1.34 ± 0.34 s(-1), P=0.3). S did not change with dobutamine infusion (-17.7 ± 3.4% versus -18.4 ± 3.9%, P=0.24), whereas SR increased significantly (-1.30 ± 0.29 versus -2.26 ± 0.49 s(-1), P<0.001). RV Ees correlated with SR (rs= -0.47, P<0.001), but not with S (rs=0.07, P=0.5) or other echocardiographic parameters. CONCLUSIONS: In contrast to S, SR was not affected by preload and correlated with Ees of the systemic RV. SR may be a useful noninvasive surrogate of RV contractility and suitable for follow-up of patients with hypoplastic left heart syndrome after Fontan palliation.

Does left ventricular size impact on intrinsic right ventricular function in hypoplastic left heart syndrome?

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index ≤ 1.33 cm(2)/m(2), n=29; group 2: LV area index>1.33 cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70 ± 1.92 vs. 3.68 ± 2.68 mmHg/ml; Mw: 52.75 ± 14.98 vs. 51.09 ± 16.63 mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40 ± 0.26 vs. 0.52 ± 0.45; group 2: 0.68 ± 0.44 vs. 0.47 ± 0.38 mmHg/ml, P<0.01). Furthermore Eed was lowest in patients with mitral atresia/aortic atresia, the anatomic subgroup with the smallest LV remnant. CONCLUSIONS: Intrinsic systolic RV function is not affected by the size of the hypoplastic LV in survivors of surgical palliation of HLHS. Diastolic stiffness, however, was higher in patients with a larger LV remnant.

Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome.

OBJECTIVE: The Norwood procedure, the first surgical step of staged palliation for hypoplastic left heart syndrome, is also applied for other complex single-ventricle lesions with systemic outflow tract obstruction or aortic arch hypoplasia. We reviewed our 15-year institutional experience with the Norwood procedure for patients with and without hypoplastic left heart syndrome. METHODS: A total of 41 patients without hypoplastic left heart syndrome and 212 patients with hypoplastic left heart syndrome who underwent a Norwood procedure between January 1996 and December 2010 were enrolled. Full medical records were reviewed to assess the determinants of outcome. RESULTS: Early failure (death or cardiac transplantation) was 7% in patients without hypoplastic left heart syndrome and 13% in patients with hypoplastic left heart syndrome (P = .29). Frequency of postoperative complications, duration of postoperative ventilation, and length of vasoactive drug treatment were not different between groups. Transplant-free survival until the second operative step trended to be higher for patients without hypoplastic left heart syndrome (92% vs 80%, P = .067). Recurrent aortic arch obstruction was more common in patients without hypoplastic left heart syndrome (15/39 vs 32/171, P = .008), but there were 4 patients with stenosis of the proximal aortic arch. In subsequent procedures, 31 patients without hypoplastic left heart syndrome underwent superior cavopulmonary anastomosis and 5 biventricular repair. Overall transplant-free survival was not different between groups (P = .119) but trended to be higher in patients with a systemic or substantial left ventricle remnant contributing to cardiac output (P = .082). CONCLUSIONS: Early and long-term survivals and postoperative complications were similar between patients with and without hypoplastic left heart syndrome undergoing a Norwood operation. Recurrent aortic arch obstruction was common in both groups but more prevalent in patients without hypoplastic left heart syndrome.

Angiographic evaluation of the coronary artery anatomy in patients with hypoplastic left heart syndrome.

OBJECTIVES: Evaluation of coronary anatomy in survivors of staged palliation for hypoplastic left heart syndrome might be capable of identifying anomalies and morphologic changes of the coronary arteries that may have implications for outcome. METHODS: We reviewed coronary angiograms obtained by native aortic root injection of 84 patients (mitral atresia/aortic atresia, n = 39; mitral stenosis/aortic stenosis, n = 25; mitral stenosis/aortic atresia (MS/AA), n = 13 and mitral atresia/aortic stenosis, n = 7). Epicardial course, anomalies, coronary dominance and native ascending aorta dimensions were analysed. RESULTS: Right dominance was present in 51%, left in 37% and balanced type in 12%. Dominance was unrelated to anatomic subtypes (P = 0.16). Ventriculocoronary connections (VCCs) were found in 15 (18%) and tortuosity was seen in 28 (33%) patients. Both occurred more often with MS/AA (tortuosity: 12 of 13 patients, P < 0.001; VCCs: 6 of 13 patients, P = 0.001). Collaterals to extra-cardiac vessels were visualized in 41 (49%) patients. Native ascending aorta dimensions were smaller with aortic atresia [40 (18-107) vs. 127 (32-328) mm(2)/m(2), P < 0.001]. In 18 patients with a relatively large native aorta [106 (36-328) vs. 44 (18-248) mm(2)/m(2), P < 0.001] retention of contrast in the aortic root identified areas of low blood flow. CONCLUSIONS: Left coronary dominance was more prevalent compared with the normal population. The impact of observed anomalies is unclear. Most VCCs are small and probably have no impact on coronary perfusion. Native ascending aorta dimensions were larger in patients with aortic stenosis. Larger aortic roots may predispose to thrombus formation and effective anticoagulation might be considered.

Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome.

OBJECTIVES: While hospital mortality after the Norwood operation for hypoplastic left heart syndrome (HLHS) has decreased steadily, interstage mortality until the superior cavopulmonary anastomosis (SCPA) remains a major concern. Our aim was to institute a home surveillance programme to decrease interstage mortality. METHODS: We enrolled 45 HLHS patients surviving the Norwood operation into our home surveillance programme and compared them with 97 patients treated before the initiation of the programme and with a third group of 20 patients not discharged between the first- and the second-stage operation. While still in hospital, parents were taught to record weight and fluid intake as well as oxygen saturations with the help of a vital sign monitor. During the last week of the hospital stay, the following criteria had to be met: oxygen saturation >75%, weight gain of at least 20-30 g in 3 days and a maximum weight loss of 30 g in a day. After discharge, these criteria had to be maintained at all times or the parents were supposed to call our hospital. Additionally, an experienced paediatric cardiologist from our centre called the parents at home once a week. RESULTS: Interstage mortality was reduced significantly from 12.4% (12/97) to 2.2% (1/45) (P = 0.042). The number of patients, who were not discharged before the SCPA, was significantly higher after the start of the home surveillance programme (12/57 vs. 8/105, P = 0.022). After discharge, 14 (31%) infants breached the surveillance criteria. Of these, one patient died and eight patients were operated earlier (SCPA, n = 6; shunt replacement, n = 2). The remaining five patients could be discharged home after observation. Children in the home surveillance programme were younger [102 (67-299) vs. 152 (77-1372) days, P = 0.001] and weighed less (5.09 ± 0.79 vs. 5.75 ± 1.22 kg, P = 0.001) at the SCPA compared with the remainder. Early survival after SCPA was not different. CONCLUSIONS: The home surveillance programme led to an important decrease in interstage mortality. The adherence to the surveillance criteria before discharge resulted in a larger number of patients receiving inpatient treatment until SCPA. Earlier SCPA in the surveillance group had no negative impact on early survival after SCPA.

Perioperative cerebral and somatic oxygenation in neonates with hypoplastic left heart syndrome or transposition of the great arteries.

OBJECTIVE: Significant depression in cerebral oxygen saturation has been observed in patients with hypoplastic left heart syndrome (HLHS) undergoing Norwood operations. We monitored cerebral oxygen saturation with near-infrared spectroscopy before and after this procedure. Patients with transposition of great arteries (TGA) before and after arterial switch operation were also studied to elucidate whether post-cardiopulmonary bypass (CPB) changes in cerebral oxygen saturation are related to CPB or hemodynamic alterations inherent in single-ventricle physiology. METHODS: We monitored 33 patients with HLHS and 20 with TGA 24 hours before and 48 hours after CPB. In addition to cerebral oxygen saturation, routine measurements of oxygen transport and delivery were performed. RESULTS: Preoperatively, cerebral oxygen saturation was higher in patients with HLHS than with TGA (61% ± 7% vs 56% ± 8%, P = .04). After CPB, cerebral oxygen saturation was markedly depressed in both groups but increased toward end of monitoring (HLHS vs TGA minimal value 42% ± 12% vs 54% ± 11%, P < .001, value 48 hours after CPB 62% ± 7% vs 80% ± 8%, P < .0001). Routine measures of oxygen delivery, such as arterial and central venous oxygen saturations, were similar at minimal cerebral oxygen saturation and 48 hours after CPB. CONCLUSIONS: Depression of cerebral oxygen saturation is prevalent among neonates with congenital heart disease regardless of whether univentricular or biventricular circulation is present, suggesting that cerebral desaturation is mainly induced by CPB's effect on cerebral blood flow. Routine measures of oxygen delivery fail to indicate cerebral desaturation.

The impact of afterload reduction on the early postoperative course after the Norwood operation - a 12-year single-centre experience.

OBJECTIVE: The objective of this study was to analyse the postoperative course and early outcome after the Norwood operation for patients with hypoplastic left heart syndrome. We particularly aimed to assess the impact of surgical and pharmacological modifications introduced. METHODS: Of 157 patients who underwent the Norwood operation between January 1996 and December 2007, postoperative intensive care data on haemodynamics, pharmacological support and ventilation were analysed from 146 patients (six patients died intra-operatively and data were incomplete in five). The cohort was divided into three groups depending on the surgical method and type of afterload reduction. Patients of group 1 (n=39, January 1996-December 1999) were operated with deep hypothermic circulatory arrest. In patients of group 2 (n=59, January 2000-June 2003) and group 3 (n=59, July 2003-December 2007) antegrade selective cerebral perfusion was used. Patients of groups 1 and 2 received sodium nitroprusside to reduce afterload; in group 3 phentolamine was used. RESULTS: There were no differences between the groups in terms of preoperative status and anatomy, except a higher incidence of prenatal diagnosis between groups 3 and 1. The duration and dosage of sodium nitroprusside administration were similar in groups 1 and 2. The median duration of afterload reduction was significantly longer in group 3 compared with both the other groups (72 h (range: 24-201 h) vs 48 h (range: 8-145 h) and 48 h (range: 4-173 h), respectively). The median ventilation times was shorter in group 2 compared with group 1 (61 h (range: 16-1191 h) vs 119 h (range: 26-648 h)). During the first 36 postoperative hours, the mean arterial blood pressure and coronary perfusion pressure were significantly lower in group 3 than in group 1 (50.7+/-4.8 and 28+/-3.7 mmHg vs 53.6+/-5.2 and 31.4+/-4.3 mmHg), but, in patients of group 3, the time period to consistently reach a mean arteriovenous oxygen difference below 5 ml dl(-1) was markedly shorter than in the other groups (group 3: 12h 4.90+/-1.97 ml dl(-1); group 1: 24h 4.53+/-2.25 ml dl(-1) and group 2: 24h 4.57+/-2.04 ml dl(-1)). Complication rates were similar between the groups. However, 30-day mortality decreased over the study period to an exponentially weighted moving average of 2.3%. CONCLUSION: Adamant afterload reduction improves systemic blood flow early after the Norwood operation and may have contributed to the reduction in early postoperative mortality achieved over 12 years.