Impact of Optimal Fenestration Size on Outcomes of High-Risk Fontan Patients.

BACKGROUND: We aimed to analyze mid-term outcomes of the fenestrated Fontan procedure, focusing on the fenestration size. METHODS: We retrospectively reviewed the outcomes of the fenestrated Fontan procedure. Among 165 patients who underwent the Fontan procedure from 2011 to 2021, fenestration was created in 27 patients with the highest risks, including those with hypoplastic left heart syndrome, hypoplastic pulmonary arteries, heterotaxy syndrome with high pulmonary vascular resistance, and pulmonary arterial pressure >15 mm Hg. The patients underwent the procedure at a median age of three years (body weight, 11.4 kg; body surface area, 0.54 m2). Fenestration sizes were 3.5 to 5 mm. RESULTS: Spontaneous fenestration closure occurred within one year postoperatively in nine patients. Among them, three experienced Fontan failure, necessitating refenestration. Although fenestration size did not differ, the size corrected by body surface area at the time of surgery was smaller in patients with fenestration closure (6.4 mm/m2 vs 8.3 mm/m2, P < .05). Patients with a fenestration <7 mm/m2 were more likely to have fenestration closure within one year postoperatively, and those with a fenestration >9 mm/m2 were more likely to have severe desaturation and require home oxygen therapy following discharge. CONCLUSIONS: Spontaneous fenestration closure affected the frequency of Fontan complications. A very small fenestration size corrected by body surface area was a significant risk factor for spontaneous closure. Conversely, a very large fenestration size corrected by body surface area resulted in severe desaturation. The optimal fenestration size to prevent early spontaneous closure and severe desaturation is approximately 8 mm/m2.

Pulmonary artery coarctation repair in univentricular heart: indications and optimal timing.

BACKGROUND: To analyse surgical outcomes of pulmonary artery coarctation in univentricular hearts, focusing on surgical indications and optimal timing. METHODS: We retrospectively reviewed 49 patients with pulmonary artery coarctation in univentricular hearts treated at our institution between 1993 and 2022. Twenty-eight patients were diagnosed before first-stage palliation. Of these, 14 underwent systemic-pulmonary shunt only as first-stage palliation (Group 1), and 14 underwent systemic-pulmonary shunt plus surgical pulmonary artery plasty as first-stage palliation (Group 2). Twenty-one patients diagnosed after first-stage palliation underwent surgical pulmonary artery plasty at the time of bidirectional Glenn procedure (Group 3). RESULTS: Follow-up period after initial palliation was 6±8 years. The Fontan procedure was successful in 35 patients (71%) aged 28±26 months (range 18-139). Freedom from interstage death (Group 1, 53%; Group 2, 85%; Group 3, 93%) and interstage reintervention (Group 1, 50%; Group 2, 75%; Group 3, 73%) rates were significantly lower in Group 1 (p = 0.01). Five and four patients in Group 1 and Group 3, respectively, needed additional shunts before the bidirectional Glenn procedure. In Group 1, one patient with a non-confluent pulmonary artery achieved hemi-lung Fontan circulation. In Group 2, one patient suffering with a non-confluent pulmonary artery could not achieve Fontan circulation, whereas another patient with pulmonary venous obstruction achieved hemi-lung Fontan circulation. CONCLUSIONS: Surgical pulmonary artery plasty performed at first-stage palliation improved outcomes of pulmonary artery coarctation in univentricular hearts, particularly when pulmonary artery coarctation had already progressed during the neonatal period or early infancy.

Long-term outcomes of functional single ventricles associated with heterotaxy syndrome†.

OBJECTIVES: The goal of this study was to determine the long-term surgical outcomes of patients with functional single ventricles associated with heterotaxy syndrome, risk factors for mortality and factors associated with Fontan stage completion. METHODS: Overall, 279 patients with a functional single ventricle associated with heterotaxy syndrome who underwent an initial surgical procedure at our institute between 1978 and 2021 were grouped into 4 "eras" based on the surgical year during which the initial procedure was performed: era 1 (1978-1989, n = 71), era 2 (1990-1999, n = 98), era 3 (2000-2009, n = 64) and era 4 (2010-2021, n = 46). Neonatal surgery was more frequent in eras 3 and 4 than in eras 1 and 2. RESULTS: Overall, 228 patients had right atrial isomerism; 120 patients (43.0%) had a total anomalous pulmonary venous connection; and 58 patients (20.8%) underwent an initial procedure as neonates. Overall survival rates at 10, 20 and 30 years after the initial procedure were 47.1%, 40.6% and 36.1%, respectively. Neonatal surgery (P < 0.001), total anomalous pulmonary venous connection repair at the initial procedure (P < 0.001) and early era (P < 0.001) were identified as risk factors for mortality, with the last 2 variables being negatively associated with Fontan stage completion (P < 0.001 for both). CONCLUSIONS: Although era had a favourable effect on survival, total anomalous pulmonary venous connection with intrinsic pulmonary vein obstruction was associated with both mortality and Fontan stage completion. CLINICAL REGISTRATION NUMBER: R19092.

Norwood Operation with Right Ventricular-Pulmonary Artery Shunt Versus Comprehensive Stage II After Bilateral Pulmonary Artery Banding Palliation.

As a strategy for the primary Norwood operation, the right ventricular-pulmonary artery shunt is associated with satisfactory early outcome. However, use of this shunt after bilateral pulmonary artery banding remains controversial. This study compared the operative outcomes and late hemodynamics in patients who underwent the Norwood operation, preceded by bilateral pulmonary artery banding, with a right ventricular-pulmonary artery shunt or with bidirectional Glenn anastomosis (comprehensive stage II strategy). We retrospectively reviewed 38 patients who underwent the Norwood operation preceded by bilateral pulmonary artery banding between 2004 and 2017. Of these, 17 underwent the Norwood operation with a right ventricular-pulmonary artery shunt (Group S), whereas 21 underwent the comprehensive stage II strategy (Group G). 5 years after the Norwood operation, 10 (60%) and 17 (81%) patients in Group S and Group G, respectively, underwent the Fontan procedure. Group S showed significantly lower pressure in the superior vena cava after bidirectional Glenn anastomosis than Group G (13 ± 2 mmHg vs. 18 ± 3 mmHg; p < 0.01), but pressures were similar after the Fontan procedure. The right ventricular end-diastolic volume at 1 year post-Fontan procedure was significantly higher in Group S than in Group G (142 ± 41% vs. 91 ± 28%; p < 0.01). In terms of early outcomes, the Norwood operation with a right ventricular-pulmonary artery shunt enabled low pressure in the superior vena cava, but in the long term, this shunt adversely influenced the right ventricular volume.

Left bronchial compression after aortic arch reconstruction for coarctation of the aorta.

BACKGROUND: This study aimed to investigate the preoperative positional relationships between the blood vessels and the left bronchus during the development of left bronchial compression (LBC) after aortic arch reconstruction for coarctation of the aorta (CoA). METHODS: We retrospectively reviewed data from 29 patients with CoA who underwent aortic arch reconstruction via median sternotomy between 2009 and 2019. The patients were divided into those who underwent aortic arch advancement (AAA) with (C group, six patients) or without (N group, 10 patients) postoperative LBC and those who underwent extended end-to-end anastomosis (E group, 13 patients). We determined the sterno-spinal axis (SSA) of the patients and evaluated the preoperative and postoperative positions of the vessels on the axial and three-dimensional multidetector row computed tomography (CT) angiographic images. RESULTS: The distance between the ascending and descending aortas was significantly smaller in both C and N groups postoperatively than preoperatively. The distance between the descending aorta and SSA in the C group was markedly smaller compared with that in the other groups preoperatively and was increased postoperatively. Preoperatively, the distance from the left bronchus to the aortic arch in the C group was significantly larger than that in the other groups and was decreased postoperatively. The postoperative arch angle in the C group was significantly smaller than that in the other groups. CONCLUSION: A preoperative midline position of the descending aorta and highly displaced aortic arch in relation to the left bronchus on preoperative CT images could cause the postoperative LBC after AAA.

Congenital Non-Confluent Pulmonary Artery Sourced from Bilateral Arterial Ducts: A Rare Anomaly.

This study aimed to identify the influence of the non-confluent pulmonary artery originating from the bilateral arterial ducts (AD) on the outcomes of the Fontan circulation. We retrospectively reviewed the records of nine patients with bilateral AD and a non-confluent pulmonary artery in a single ventricle at our institution between 1993 and 2023. Three patients showed maintained AD or underwent a systemic-pulmonary shunt for stenotic AD, followed by the Glenn procedure. Four patients underwent systemic-pulmonary shunt with angioplasty as the first palliation, followed by the Glenn procedure. Two patients underwent systemic-pulmonary shunt or AD stenting as the first palliation and systemic-pulmonary shunt with angioplasty as the second palliation. There were no cases of interstage mortality. Pulmonary arteries grew and achieved a good balance (pre-Fontan pulmonary artery index [PAI], 164 ± 27 mm2/m2; right/left PAI ratio, 1.06 ± 0.23). All patients underwent the Fontan procedure (median, 3.5 years; range, 2.3-6.4 years) and were followed up for 7.8 years (range, 0.1-16.4 years) after the procedure. One patient required hospitalization for heart failure at 1.8 years, and three patients required catheter intervention for pulmonary stenosis within 2 months after the Fontan procedure. Non-confluent pulmonary arteries originating from the bilateral AD do not preclude Fontan completion and good Fontan outcomes by restoring balance to pulmonary blood flow. Due to the limited sample size in this study, additional research is imperative to delve deeper into our findings and enhance understanding of the most effective surgical approach for this disease.

Outlet Ventricular Septal Defect: Impact of Surgery on the Late Aortic and Pulmonary Valve Functions.

This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7 years (interquartile range: 0-17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery were factors associated with post-operative residual aortic regurgitation. Mild pulmonary regurgitation was observed in 12%, 30%, and 40% of patients 5, 10, and 15 years after surgery, respectively. There were no significant differences in age and weight at which surgery was performed between patients with mild pulmonary regurgitation and those with less than mild pulmonary regurgitation. However, the number of sutures across the pulmonary valve was associated with post-operative pulmonary regurgitation (P < 0.01). As some patients with mild pre-operative aortic regurgitation may not improve even after surgery, early surgical intervention is necessary when aortic regurgitation appears. Some patients may develop post-operative pulmonary regurgitation in the long term, suggesting the need for careful follow-up.

Congenital Mitral Regurgitation Repair Based on Carpentier's Classification: Long-Term Outcomes.

BACKGROUND: There are few reports of the outcomes of standardized surgical management addressing the etiologic and morphologic aspects of mitral valve malformation according to Carpentier's classification. This study aimed to evaluate the long-term outcomes of mitral valve repair in children according to Carpentier's classification. METHODS: Patients who underwent mitral valve repair at our institution between 2000 and 2021 were retrospectively reviewed. Preoperative data, surgical techniques, and outcomes were analyzed according to Carpentier's classification. The proportion of patients free of mitral valve replacement and reoperation was estimated using Kaplan-Meier analysis. RESULTS: Twenty-three patients (median operative age, four months) were followed up for 10 (range, 2-21) years. Preoperative mitral regurgitation was severe in 12 patients and moderate in 11 patients. Eight, five, seven, and three patients had Carpentier's type 1, 2, 3, and 4 lesions, respectively. Ventricular septal defect (N = 9) and double outlet of the great arteries from the right ventricle (N = 3) were the most commonly associated cardiac malformations. There were no cases of operative mortality or deaths during the follow-up. The overall five-year rate of freedom from mitral valve replacement was 91%, whereas the five-year rates of freedom from reoperation were 74%, 80%, 71%, and 67% in type 1, 2, 3, and 4 lesions, respectively. Postoperative mitral regurgitation at the last follow-up was moderate in three patients and less than mild in 20 patients. CONCLUSIONS: Current surgical management of congenital mitral regurgitation is generally considered adequate; however, more complicated cases required a combination of various surgical techniques.

Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta.

Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. A retrospective comparison on the echocardiographic evaluation of 30 patients with suspected CoA between May 2016 and April 2021 was performed. The patients were divided into a postnatal surgical intervention group (n = 13) and a non-intervention group (n = 17). The measurements that showed significant differences were aortic isthmus diameter Z-score (p < 0.001), ductus arteriosus diameter/aortic isthmus diameter (p < 0.001), and distal aortic arch (DA) index (p < 0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Measurement of the DA index improved the diagnostic rate of fetal CoA and a DA index ≧ 1.28 indicated fetal CoA cases requiring surgical intervention.

Influence of Bovine Arch Anatomy on Surgical Outcomes of Coarctation of the Aorta.

This study aimed to evaluate the outcome of coarctation of the aorta (CoA) repair with a special interest in bovine arch anatomy. Fifty-six patients who underwent CoA repair between 2010 and 2021 were included in this retrospective study. Of these, 11 patients had bovine arch anatomy. Surgical outcomes were reviewed. Computed tomography was used to analyze aortic arch geometry for all cases preoperatively. The gap between anastomotic sites was calculated at the linear region of the lesser curvature of the aortic arch between the distal ascending aorta and the proximal descending aorta. CoA repair was performed at a median age of 39 days (median body weight 3.3 kg). Thirty-two patients underwent extended direct anastomosis, 22 patients underwent direct anastomosis, and two patients underwent lesser curvature patch augmentation. The median follow-up period was 47 months. There were no early deaths. In patients who underwent direct and extended direct anastomosis, nine recoarctation and one left pulmonary venous obstruction events occurred. Moreover, freedom from these adverse events was 81% in normal arch and 50% in bovine arch patients at 10 years (P = 0.04). Two patients with a bovine arch anatomy who underwent lesser curvature patch augmentation had good outcomes. The distal arch was narrower and longer, and the gap between anastomotic sites was longer in patients with a bovine arch anatomy than with a normal arch (P < 0.01). In CoA with a bovine arch anatomy, the gap between anastomotic sites was long. This adversely influenced the outcomes of the CoA repairs.

Bilateral pulmonary artery banding for transposition of the great arteries complex with coarctation.

Surgical approaches for transposition of the great arteries with aortic arch obstruction include primary repair and two-stage repair. However, neither approach provides a satisfactory outcome. We report a case of patient who underwent two-stage repair, wherein arterial switch operation combined with aortic arch reconstruction was preceded by bilateral pulmonary artery banding; this yielded good outcomes. This approach safely avoids primary repair in the neonatal period and allows for the opportunity to evaluate right ventricle outlet tract stenosis before the definitive repair.

Management of neonatal cardiac rhabdomyoma obstructing the aortic valve.

Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative two-dimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

Coarctation of Aorta With Circumflex Aorta: Risk of Bronchial Compression and Recoarctation.

Coarctation of the aorta with a circumflex aorta is a very rare congenital heart anomaly. This report describes the treatment of 3 patients with this condition. All patients underwent direct anastomosis for aortic arch repair. However, postoperatively, in 2 patients, left main bronchial compression with gradual traction of the descending aorta toward the left side developed. The third patient experienced recoarctation of the aorta resulting from excessive tension. Surgical strategies for coarctation of the aorta should consider the positional relationship of the descending aorta and aortic arch, such as cases of a circumflex aorta.

Aortic-to-Right Ventricle Shunting for Rare Cardiovascular Conditions.

Pulmonary atresia with hypoplastic right ventricle and ventricular septal defect with right ventricle-dependent coronary circulation are very rare congenital heart anomalies. This report describes the case of a patient in whom aortic-right ventricle shunting was surgically established with a satisfactory postoperative course. Aortic-right ventricle shunting can enhance oxygenation of the sinusoidal communication and reduce the incidence of myocardial ischemia without influencing the left ventricular volume load through the ventricular septal defect despite preservation of the septal defect.

Reconstruction in left main coronary artery atresia with bypass graft obstruction.

Paediatric coronary artery bypass surgery with internal thoracic artery grafting is the optimal choice for left main coronary artery atresia; we report successful reconstruction in a patient with bypass graft obstruction. The pulmonary trunk was transected to expose the left main coronary stem, which was opened beyond the obstruction and the incision extended through the left main coronary artery to the circumflex bifurcation and left anterior descending artery. A funnel-shaped coronary ostium was created with a glutaraldehyde-treated autologous pericardium onlay patch. Surgical reconstruction is a good alternative for left main coronary artery atresia with graft obstruction and preserves the remaining internal thoracic artery for future use.

Anticoagulation Therapy After the Fontan Procedure.

The optimum postoperative anticoagulation therapy type and duration after the Fontan procedure remains unclear. This study aimed to evaluate whether our approach of administering only oral antiplatelets without warfarin due to serious bleeding complication risks in children is reasonable. We retrospectively reviewed the data of 249 patients who underwent the Fontan procedure. Total cavopulmonary connection with extracardiac conduit was performed in 230 patients (92%), the lateral tunnel Fontan procedure was performed in 18 patients (8%), and intraatrial conduit Fontan procedure was performed in one patient. Aspirin administration (initial dose: 5 mg/kg/day) was continued in all patients. Only 29 patients (11%) received combined administration of aspirin and warfarin for various reasons, and warfarin was discontinued in 20 (71%) of these within a median of 16 months postoperatively. Sixteen patients at high risk of thromboembolism were started on warfarin. Hospital mortality was 0%, with no thromboembolic or bleeding event-related early death. Nine late deaths, not associated with bleeding nor thromboembolism, occurred except one resulting from cardiac infarction 1.9 years after the procedure. During hospitalization, five patients experienced graft thromboembolism, two of which underwent graft exchange, and the others were started on warfarin. In the late phase (3.1 ± 6.6 years), cerebral infarction (n = 3), peripheral pulmonary artery occlusion (n = 2), gastrointestinal bleeding (n = 5), and respiratory hemorrhage (n = 4) occurred. Eight of nine patients with bleeding events received only aspirin. Thromboembolic events occurred in six patients within the first year after the procedure. Bleeding events occurred at 8 ± 5.5 years after the procedure. Expected freedom from a thromboembolic event was 99.8% at 1 year and 93.4% at 20 years. Expected freedom from a bleeding event was 100% at 1 year and 88.4% at 20 years. In children at low risk of thromboembolism after the Fontan procedure, continuous administration of an oral antiplatelet agent alone without warfarin seems to represent a reasonable prophylactic approach.

Norwood procedure for hypoplastic left heart syndrome associated with valvular pulmonary stenosis.

Association between hypoplastic left heart syndrome and valvular pulmonary stenosis is very rare. Severity of valvular pulmonary stenosis in this setting limits management options. Consequently, patients with this condition are considered poor candidates for Norwood stage one reconstruction. Herein, we describe a newborn with hypoplastic left heart syndrome and significantly dysplastic pulmonary valve who successfully underwent the Norwood procedure with neoaortic valve reconstruction. Therefore, the Norwood procedure with neoaortic valve reconstruction might be an option for this difficult condition.

Fontan operation for tricuspid atresia with absent pulmonary valve: a case series.

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Surgical treatment of isolated unilateral absence of pulmonary artery with pulmonary hypertension.

A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

Active mitral valve endocarditis with widespread interventricular septal abscesses in a paediatric patient.

We present the case of a previously healthy 2-year-old boy with extensive infective mitral valve endocarditis, with 2 huge, mobile vegetations attached to the anterior leaflet of the mitral valve and to the left ventricular outflow tract, and interventricular septal abscesses extending into the left ventricular outflow tract without any septal defects. He underwent mitral valve repair and simultaneous drainage of the interventricular septal abscesses excluding the inlet portion to avoid postoperative complications.