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.

Preoperative Feeding in Single Ventricle Neonates is Predictive of Shorter Time to Goal Feed

Background:Patients with single ventricle anatomy are at increased risk of growth failure and malnutrition.Amongst cardiac centers,there is little standardization of feeding practices in this complex population.We hypothesized that initiation of our center’s preoperative feeding protocol would result in decreased gastrostomy tube(G-tube)use,decreased length of stay and would not result in increased Necrotizing Enterocolitis(NEC)rates.Methods:A single institution review of 52 patients who had undergone stage I single ventricle palliative repair was performed.Patient diagnoses were hypoplastic left heart syndrome(39%),atrioventricular canal(15%),and other(46%).Postoperative parameters such as time to goal feed and need for gastrostomy tube(G-tube)were compared among preoperatively fed and non-preoperatively fed groups.Time to goal feed was calculated as time from first postoperative enteral feed to goal volume of 100 mL/kg.Results:Of the 26 patients who met inclusion criteria for preoperative feeding,22 patients(85%)were fed prior to surgery.Cox proportional hazard ratio revealed that age at surgery(p=0.047)and being preoperatively fed(p=0.001)were associated with reaching goal feed sooner.Multivariable analysis revealed that being preoperatively fed made a patient twice as likely to reach goal feed sooner(p=0.047).Univariable logistic regression revealed that days on total parenteral nutrition(p=0.018),length of hospitalization(p=0.008),and time to 1st postoperative feed(p=0.020)were significantly associated with higher odds of needing a G-tube postoperatively.Multivariable logistic regression did not show any predictors of postoperative G-tube usage.However,there was a trend towards lower G-tube usage in the preoperatively fed group.Conclusions:Implementing a standardized preoperative feeding protocol in single ventricle neonates can result in significantly shorter time to goal feed in preoperatively fed patients.It is beneficial for institutions to begin implementing standard feeding protocols to improve nutrition and growth outcomes.

Effectiveness of Bilateral Pulmonary Artery Banding in Patients with Hypoplastic Left Heart Syndrome and Congenital Heart Defects with a Functional Single Ventricle: A Single-Center Retrospective Study

Background:Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome(HLHS)and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period,especially in severely unstable patients.In our study we compared different surgical techniques of bilateral pulmonary artery banding(PAB)in respect to their success in balancing systemic and pulmonary blood flow.Methods:We included 44 neonates with a HLHS and congenital heart diseases(CHD)with a functional single ventricle underwent a hybrid operation:bilateral PAB and patent ductus arteriosus stenting.The hybrid surgery for method No.1 is performed as a one-stage procedure,together with patent ductus arteriosus(PDA)stenting.After median sternotomy,two Gore-Tex 1–2 mm wide bands with a diameter of 3–3.5 mm are put.When we apply method No.2 then the thread is used to create bands.Method No.3 is distinguished by intraoperative assessment of blood flow at the site of narrowing of the branches of the pulmonary artery and optional stenting of the PDA.The cuff for banding is made of Gore-Tex tubing.Effectiveness when applying method Nos.1 and 2 is assessed by the change in invasive blood pressure and oxygen saturation after narrowing of the branches of the pulmonary artery.Also,with these techniques PDA stenting by inserting the introducer via pulmonary artery trunk is performed.Results:HLHS with mitral or aortic valve atresia or both was present in 19 patients(43.1%),with severe left heart obstruction resulting in PDA dependent systemic circulation in 16 babies(36.4%).CHD with single ventricle physiology occurred in 9 patients(20.5%).14 babies(31.8%)undergo the procedure following the method No.1,8 patients(18.2%)method No.2 and 22 patients(50%)method No.3.Qp/Qs=1/1 was achieved in 30 patients(30/44,68.1%):as a result of the method No.1 was achieved in 5 patients(5/14,35.7%),method No.2 in 4 patients(4/8,50%),method No.3 in 21 patients(21/22,95.5%).Multivariate regression analysis revealed that method No.3 significantly increases the chances of hemodynamic efficacy operations(OR=35.0;p=0.005;CI(95%)3–411.5).Conclusion:Application of the operation technique No.3 in combination with the intraoperative assessment of blood flow parameters at the site of banding of the branches of the pulmonary artery are the most optimal criteria for achieving Qp/Qs=1/1.If there are signs of restriction at the level of the foramen ovale,atrioseptostomy should be done in the second stage after bilateral pulmonary banding.

Comparison of Intracardiac and Extracardiac Malformations Associated with Single Atrium,Single Ventricle and Single Atrium-Single Ventricle Using Dual-Source Computed Tomography

Background:To evaluate the qualitative and quantitative differences between intracardiac and extracardiac vascular malformations in patients with a single atrium(SA),single ventricle(SV)and single atrium-single ventricle(SA-SV)using dual-source CT(DSCT),and to compare the diagnostic performances of DSCT and transthoracic echocardiography(TTE).Methods:This retrospective study included 24 SA,75 SV and 24 SA-SV patients who underwent both DSCT and TTE before surgery.The diagnostic values of DSCT and TTE for intracardiac and extracardiac malformations were compared according to the surgical results.The diameters of the major artery and vein were measured and calculated based on DSCT and compared among the three groups.Results:The most common malformation was pulmonary artery disease in SA(50.0%)and SA-SV(45.8%)groups and patent ductus arteriosus(33.3%)in SV group.Although there was no statistical difference,arterial development was relatively poor in the SA group.All groups showed the trend of pulmonary artery stenosis(SA vs.SV vs.SA-SV:50.0%vs.30.7%vs.33.3%).There was a significant difference in mean pulmonary vein index among the groups(p=0.017).The diagnostic sensitivity of DSCT was superior to that of TTE for extracardiac malformations.Conclusions:The most common malformation in SA and SA-SV patients is pulmonary artery stenosis.SV patients are most likely to be complicated with patent ductus arteriosus.DSCT is more advantageous than TTE for diagnosing combined extracardiac malformations and can accurately measure the diameter of arteriovenous vessels.

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad.In patients with congenital heart disease,one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure(CVP).In patients with single-ventricle physiology,the second stage of palliation includes bidirectional Glenn,which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle.Typically,early in the post-operative period,patients may experience a headache due to the acute increase in CVP,and symptoms improve over time.Idiopathic intracranial hypertension(IIH),also known as pseudotumor cerebri,is a rare neurologic disorder in children,characterized by raised intracranial pressure(ICP)in the absence of brain parenchymal lesions or cerebrospinal fluid(CSF)abnormalities.While the pathogenesis of IIH is unknown,early recognition and treatment of IIH are important to prevent permanent vision loss.There are only rare reports of IIH in patients with Fontan circulation.To our knowledge,we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

Background:The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow(AnPBF)in single-ventricle(SV)and interrupted inferior vena cava(IVC)heart disease is still uncertain as yet.Methods:We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018,perioperative,operative and postoperative characteristics were recorded.Results:A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years(range 0.5–24.7 years),of which 12(66.7%)were male and 6(33.3%)were female.The mean saturation was 76.2±8.5%in preoperative period and 94.2±2.2%in postoperative period.All patients had kept AnPBF.The median duration of mechanical ventilation was 12 h(range 2.5–22.5 h)and the median duration of pleural drainage was 5 days(range 2–27 days).The median hospital stay was 9 days(range 6–70 days).There was no operative death and no mortality was seen in early postoperative period.Follow-up was 100%completed,with an average follow-up period of 6.1±2.7 years(range 1–11 years).4 patients died during the follow-up.The overall 5 and 10 years’survival rates estimated by Kaplan-Meier method were 88%and 68%,respectively.Although there were no significant differences in the duration of postoperative follow-up between the death group and the survival group(p>0.05),the major systemic ventricular end-diastolic diameter(SVEDD)(p=0.018)and the degree of AVVR(p=0.001)showed significant difference between the two groups.The diameters of main pulmonary artery showed significant growth in both the death group(p=0.015)and the survival group(p=0.012)over time.SVEDD had no significant increase in the survival group(p=0.665)but was significantly larger in the death group(p=0.014).Multivariable risk factors of late mortality in patients treated with Kawashima procedure were follow-up AVVR(p=0.044;HR:3.124;95%CI:1.030–9.473)and SVEDD(p=0.031;HR:9.766;95%CI:1.226–77.8).14 patients(100%)were all in New York Heart Association(NYHA)functional class I and the mean saturation was 93±2%at last follow-up.Only one patient finished Fontan completion.Conclusions:The Kawashima procedure with AnPBF can be safely performed with acceptable early and long outcomes.Although some previous studies have shown the risk of pulmonary arteriovenous malformations(PAVMS)after Kawashima procedure in the mid-and long-term,our findings are in contradiction with it.No PAVMs occurred in all the survivors.Kawashima procedure with open AnPBF may be a good option for unsuitable Fontan candidates.

Surgical outcomes of functional single ventricle with total anomalous pulmonary venous connection

Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the results of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were retrospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 patients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pulmonary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC repair had lower mortality(30.0%)and higher total cavopulmonary connection completion rate(20.0%)than those without TAPVC repair[75.0% and 0%,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0%,77.1% and 77.1%,respectively. Multivariate COX regression analysis detected that SP shunt(adjusted odds ratio:6.51;95%CI:1.26-33.71,P=0.030)is the only risk factor for survival. The reintervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1%vs. 4.3%,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warranted to support our finding.

A Ring-Reinforced Right Ventricle to Pulmonary Artery Conduit Is Associated with Better Regional Mechanics after Stage I Norwood Operation

Background:The right ventricle to pulmonary artery conduit(RVPAC)may impair right ventricular(RV)function in patients with functional single right ventricles.Modification of the RVPAC using a ring-reinforced end with dunked insertion into the RV through a limited ventriculotomy may reduce the impact on RV function.We compared RV segmental strain between patients with a traditional RVPAC and ring-reinforced RVPAC using feature tracking cardiovascular magnetic resonance(CMR)imaging.Methods:Patients with CMR examinations after Stage I operation with RVPAC between 2000 and 2018 were reviewed.Ventricular mass,volumes,late gadolinium enhancement(LGE),and peak radial and circumferential strain of the 4 segments near the RVPAC insertion site were analyzed.Results:The study included 71 CMR examinations in 61 patients(30 traditional RVPAC,31 ring-reinforced RVPAC).Prior to Stage II,the ring-reinforced RVPAC group had better peak radial strain and circumferential strain in 1 of 4 segments proximal to the RVPAC insertion site compared to the traditional RVPAC group.Prior to Stage III operation,the ring-reinforced group had better peak radial and circumferential strain in 2 of 4 segments.LGE at the RVPAC insertion site was observed in 97%of patients before Stage II and 95%of patients before Stage III.RVPAC type and regional strain were not associated with transplant-free survival during a median follow-up of 6.3 years.Conclusions:Compared to a traditional RVPAC,the ring-reinforced RVPAC is associated with improved regional mechanics at the conduit insertion site.Further investigation of long-term outcomes after ring-reinforced RVPAC is warranted.

The Glenn Shunt Revisited, A Single Center Registry in Ain Shams University Cardiology Department

Background:Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is,patients unsuitable for biventricular repair.In our country they are performed at an older age than that recommended by the literature.So,we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity.Methods:This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals.History taking including NYHA class and physical activity,clinical examination,six-minutes-walk test,laboratory investigations,full echocardiographic assessment were done for all patients.Some patients needed invasive cardiac catheterization.Results:Our registry included 178 patients who underwent bidirectional Glenn procedure referred for follow up in Ain Shams university hospital from January 2019 till July 2020.The mean age of the registry was 18.7±8.26 years(range between 5 and 37 years),85 males(48%)and 93 females(52%).Regarding the basic anatomy,double outlet right ventricle with hypoplastic left ventricle was the commonest.Furthermore,our descriptive study confirmed many characteristic similarities between our patients and patients in developing countries.Our patients underwent bidirectional Glenn shunt at a median age of 6 years which is considered a relatively old age but similar to other studies that have been made in developing countries like Pakistan,India and Iran.There is a significant delay in the operation in Egyptian patients due to lack of patients’awareness,few numbers of primary health care facilities and high economic burden.Conclusion:Although the presence of slight systemic desaturation,our study demonstrated a satisfactory functional capacity among our patients;thus,bidirectional Glenn can be considered an acceptable definite univentricular repair in patients with late presentation.

Single Stage Extra Cardiac Conduit Total Cavopulmonary Connection in Atrial Situs Inversus and Levocardia

10-year female child was presented with complex congenital heart defect—atrial situs inversus, levocardia, discordant atrioventricular connections, double outlet right ventricle, ventricular septal defect, pulmonic stenosis and straddling of atrio-ventricular valves along with bilateral superior vena cava and large patent ductus arteriosus. We describe here the strategy adopted to perform single stage fenestrated extra cardiac conduit total cavo-pulmonary connections in this patient especially concerning cardiac malposition of atrial situs inversus and levocardia.

Multi-Institutional US Experience of the Occlutech AFR Device in Congenital and Acquired Heart Disease

Objectives:To detail the US multi-institutional experience with the Occlutech©(Occlutech International AB,Helsingborg,Sweden)atrial flow regulator(AFR)in children and adults with acquired or congenital heart disease.Background:The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes,most notably pulmonary arterial hypertension,disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation.Methods:Patients were identified for inclusion from the AFR device manufacturer database.Data was collected using a RedCap database following IRB approval.8 weeks of follow up data was sought for each patient based on available data.Data was analyzed and summarized using SPSS.Results:We report the experience of 6 US centers in the implantation of AFR devices in 15 patients,across a wide age range,with different disease phenotypes and a variety of indications.Implantation was technically successful in all patients and improvement was noted in both clinical and hemodynamic parameters.There were no immediate or intermediate term complications reported.3 patients died remote from implantation.Their deaths were not felt to be related to the AFR device or related procedural complications.Conclusion:Compassionate use of the AFR device in children and adults with congenital&acquired heart disease is technically feasible and produces beneficial short term hemodynamic and symptomatic improvement.Widespread uptake of this technique and treatment at specialist centers has the potential to provide significant benefits to a variety of complex patients with currently limited treatment options and indeterminate prognosis.

Lesion-based Patterns of Morbidity and Mortality in Hospitalized Adolescents with Congenital Heart Disease

Objective:The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease(AdoCHD)from the Pediatric Health Information System(PHIS)database.Methods:The PHIS database was queried for all AdoCHD admissions aged 12–18 years(1/1/2004–12/31/2013).Major forms of CHD were identified by their International Classification of Diseases,ninth revision codes,further verified based on their secondary diagnosis and/or procedure codes.Patient characteristics,diagnoses,procedures and vital status were assessed.Results:In total,there were 4,267 adolescents admitted to 42 Children’s Hospitals,58.3%were males,24.6%single ventricle(SV)patients,64.1%bi-ventricle(BV),and 11.3%could not be classified.They accounted for 8,512 hospitalizations(41,240 total hospital days),of which 31.6%were intensive care unit(ICU)stays.ICU stay was similar for the SVand BV patients with similar duration of mechanical ventilation between the two groups.Overall,the most common CHD among in-patients was tetralogy of Fallot(TOF,36.4%).Larger proportion of the BVAdoCHD admissions were for elective surgical and electrophysiological procedures.There were 109(2.5%)heart transplantations(1.3%SV vs.0.6%BV)and 120 in-hospital deaths(2.8%)(1.1%SV vs.1.3%BV).Hypoplastic left heart syndrome was the most common diagnosis in transplanted patients(46%)and those who died(28%);TOF(29%)was frequent in 91(2.1%)patients who had cardiac arrests.Conclusions:Different hospitalization patterns exist for BV and SV AdoCHD.Recognizing this risk may encourage directing resources toward optimizing long-term care of CHD patients.

Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

Background: Patients born with a single ventricle circulation commonly experience growth failure in early life,which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectoryand clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, asystematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patientswith clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluatedby Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies.Results: Of 527 studies that underwent title and abstract screening, 15 were selected for final review. An increasedrisk of adverse post-Fontan outcomes was found for low weight patients, consistent with findings in infants.Whilst there is some evidence to suggest increased mortality in overweight adult patients, studies are conflictingas to whether overweight status is associated with increased heart failure. Increased BMI is associated with diminishedexercise capacity and deceased physiological functioning. Negative weight trajectory is associated withadverse outcomes in the peri-Fontan period, whereas a positive weight trajectory is associated with increased Fontanfailure in adulthood. Abnormal BMI (high or low) is associated with increased heart failure and poorer performancein quality-of-life scores. Conclusions: Bodyweight is a modifiable risk factor for poor clinical outcomein patients with a single ventricle circulation. Recognizing associations between bodyweight and Fontan pathophysiologymay help to define patient-centered exercise and diet interventions that minimize patient morbidityand mortality.

Acute ischemic stroke in a child with cyanotic congenital heart disease due to non-compliance of anticoagulation

BACKGROUND:Stroke is a common presentation in geriatric patients in emergency department but rarely seen in pediatric patients.In case of acute ischemic stroke in pediatric age group,management is different from that of adult ischemic stroke where thrombolysis is a good option.METHODS:We report a case of a 17-year-old male child presenting in emergency with an episode of acute ischemic stroke causing left hemiparesis with left facial weakness and asymmetry.The patient suffered from cyanotic congenital heart disease for which he had undergone Fontan operation previously.He had a history of missing his daily dose of warfarin for last 3 days prior to the stroke.RESULTS:The patient recovered from acute ischemic stroke without being thrombolyzed.CONCLUSION:In pediatric patients,acute ischemic stroke usually is evolving and may not require thrombolysis.