Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.

One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome.The cardiac malformations included dextrocardia,double outlet right ventricle,pulmonary stenosis,interrupted inferior vena cava,hemiazygos continuation and total anomalous pulmonary venous return.One-stage correction was performed.The atrial procedure consisted of intra-and extraatrial rerouting of the anomalous systemic and pulmonary venous return.The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit.The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta.The right ventricular outflow was ultimately remodeled using a valved conduit.For better perception of the complex morphology,a three-dimensional model was designed,using CT scan images.This proved to be very useful for surgical planning,especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.

Single institution experience with the Ladd's procedure in patients with heterotaxy and stage Ⅰ palliated single-ventricle

AIM:To investigate and describe our current institutional management protocol for single-ventricle patients who must undergo a Ladd's procedure.METHODS:We retrospectively reviewed the charts of all patients from January 2005 to March 2014 who were diagnosed with heterotaxy syndrome and an associated intestinal rotation anomaly who carried a cardiac diagnosis of functional single ventricle and were status post stage I palliation.A total of 8 patients with a history of stage I single-ventricle palliation underwent Ladd's procedure during this time period.We reviewed each patients chart to determine if significant intraoperative or post-operative morbidity or mortality occurred.We also described our protocolized management of these patients in the cardiac intensive care unit,which included pre-operative labs,echocardiography,milrinone infusion,as well as protocolized fluid administration and anticoagulation regimines.We also reviewed the literature to determine the reported morbidity and mortality associated with the Ladd's procedure in this particular cardiac physiology and if other institutions have reported protocolized care of these patients.RESULTS:A total of 8 patients were identified to have heterotaxy with an intestinal rotation anomaly and single-ventricle heart disease that was status post single ventricle palliation.Six of these patients were palliated with a Blaylock-Taussig shunt,one of whom underwent a Norwood procedure.The two other patients were palliated with a stent,which was placed in the ductus arteriosus.These eight patients all underwent elective Ladd's procedure at the time of gastrostomy tube placement.Per our protocol,all patients remained on aspirin prior to surgery and had no period where they were without anticoagulation.All patients remained on milrinone during and after the procedure and received fluid administration upon arrival to the cardiac intensive care unit to account for losses.All 8 patients experienced no intraoperative or post-operative complications.All patients survived to discharge.One patient presented to the emergency room two months after discharge in cardiac arrest and died due to bowel obstruction and perforation.CONCLUSION:Protocolized intensive care management may have contributed to favorable outcomes following Ladd's procedure at our institution.

An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

Abernethy malformation （AM） is a rare congenital anomaly in which the splanchnic blood bypasses the liver and drains directly into the systemic veins.It was first described by Abemethy in 1793 and has two types.Type Ⅰ is characterized by a complete portosystemic shunt and an absence of a portal vein （consists of superior mesenteric and splenic veins）,while Type Ⅱ exhibits partial portosystemic shunt and a hypoplastic portal vein.Type Ⅰ is further subclassified into subtype a and b based on the absence or presence of a patent connection between the superior mesenteric vein and the splenic vein.

Malrotation: Current strategies navigating the radiologic diagnosis of a surgical emergency

The most accurate and practical imaging algorithm for the diagnosis of intestinal malrotation can be a complex and sometimes controversial topic.Since 1900,sig-nificant advances have been made in the radiographic assessment of infants and children suspected to have anomalies of intestinal rotation.We describe the cur-rent methods of abdominal imaging of malrotation along with their pros and cons.When associated with volvulus,malrotation is a true surgical emergency re-quiring rapid diagnosis and treatment.We emphasize the importance of close cooperation and communica-tion between radiology and surgery to perform an effective and efficient diagnostic evaluation allowing prompt surgical decision making.

Clinical spectrum of primary ciliary dyskinesia in childhood

Although the triad of bronchiectasis, sinusitis and situs inversus was first described by Kartagener in 1933, the clinical spectrum of primary ciliary dyskinesia is still under investigation. Heterotaxy defects as well as upper and lower respiratory tract symptoms are the main manifestations in childhood. It is now recognized that situs inversus is encountered in only half of patients. The first lower respiratory symptoms may be present from infancy as neonatal respiratory distress. The most common lower airway manifestations are chronic wet cough, recurrent pneumonia and therapy resistant wheezing. Patients are at risk of developing bronchiectasis which may even be the presenting finding due to delayed diagnosis. Upper respiratory tract infections such as nasal congestion, nasal drainage and recurrent sinusitis as well as otologic manifestations such as otitis media or otorrhea with conductive hearing loss are also often encountered. It seems that the type of ciliary ultrastructure defects and the involved mutated genes are associated to some extent to the clinical profile. The disease, even in nowadays, is not recognized at an early age and the primary care clinician should have knowledge of its clinical spectrum in order to select appropriately the children who need further investigation for the diagnosis of this disorder.

Intestinal microbiome changes in an infant with right atrial isomerism and recurrent necrotizing enterocolitis:A case report and review of literature

BACKGROUND Necrotizing enterocolitis(NEC)is a multifactorial disease that predominantly affects premature neonates.Intestinal dysbiosis plays a critical role in NEC pathogenesis in premature neonates.The main risk factor for NEC in term infants is mesenteric hypoperfusion associated with ductaldependent congenital heart disease(CHD)that eventually leads to intestinal ischemia.The incidence of NEC in neonates with critical CHD is 6.8%-13%.However,the role of the intestinal microbiome in NEC pathogenesis in infants with ductal-dependent CHD remains unclear.CASE SUMMARY A male term neonate with right atrial isomerism underwent modified Blalock-Taussig shunt placement on the 14^(th)day of life and had persistent mesenteric hypoperfusion after surgery.The patient had episodes of NEC stageⅡA on the 1^(st)and 28^(th)days after cardiac surgery.Fecal microbial composition was analyzed before and after cardiac surgery by sequencing region V4 of the 16S rRNA gene.Before surgery,species belonging to genera Veillonella and Clostridia and class Gammaproteobacteria were detected,Bifidobacteriaceae showed a low abundance.The first NEC episode was associated with postoperative hemodynamic instability,intestinal ischemiareperfusion injury during cardiopulmonary bypass,and a high abundance of Clostridium paraputrificum(Clostridium sensu stricto I)(56.1%).Antibacterial therapy after the first NEC episode resulted in increased abundance of Gammaproteobacteria,decreased abundance of Firmicutes,and low alpha diversity.These changes in the microbial composition promoted the growth of Clostridium sensu strictoⅠ(72.0%)before the second NEC episode.CONCLUSION A high abundance of Clostridium sensu strictoⅠand mesenteric hypoperfusion may have contributed to NEC in the present case.