Outcomes of delayed chest closure after congenital heart surgery in neonates.

We present the outcomes of delayed chest closure in neonates who underwent congenital heart surgery under cardiopulmonary bypass. Eighty-one consecutive neonatal patients (age ≤ 28 days) with congenital heart diseases who underwent heart operations and after surgery, chest remained open in the intensive care unit until DCC. Correction of transposition of the great arteries pathology was the most common surgical procedure (48.1% of patients). Median sternal closure time from surgery was 3 (2-4) days. Median age of neonates was 9 (5-12) days. In addition, in 4 cases (4.9%) there was secretion from the surgical site after DCC and after taking cultures, in 2 (2.4%) of the cases a pathogen was identified. Multivariable linear regression analysis (adjusted to gender and CPB) showed that only the age-predicted the sternum closure time (ß=-0.09, 95%CI: - 0.16 to -0.02, p=0.02). In-hospital mortality was 6 (7.4%) patients. Although the DCC in neonates who underwent CHD surgical correction was related to a high mortality rate, only the age of neonates predicted the sternum closure time in the ICU.

Efficiency and Safety of Patent Ductus Arteriosus Surgical Ligation in Extremely Low Birth Weight Infants Without Chest Tube Placement.

Patent ductus arteriosus (PDA) has been associated with increased morbidity and mortality in preterm infants. Surgical ligation (SL) is generally performed in symptomatic infants when medical management is contraindicated or has failed. We retrospectively reviewed our institution's experience in surgical management of PDA for extremely low birth weight (ELBW) infants without chest tube placement assessing its efficiency and safety. We evaluated 17 consecutive ELBW infants undergoing SL for symptomatic PDA (January 2012-January 2018) with subsequent follow-up for 6 months postdischarge. Patients consisted of 9 (53%) females and 8 (47%) males. Mean gestational age (GA) at birth was 27.9 ± 2.1 weeks. Median values for surgical age (SA) from birth to operation was 10 days (interquartile range [IQR]: 8-12); PDA diameter 3.4 mm (IQR: 3.2-3.5); surgical weight (SW) 750 g (IQR: 680-850); and days of mechanical ventilation (DMV) as estimated by Kaplan-Meier curve 22 days (95% confidence interval: 14.2-29.8). We observed a statistically significant negative association between DMV and GA at birth (rho = - 0.587, p = 0.017), SA (rho = - 0.629, p = 0.009) and SW (rho = - 0.737, p = 0.001). One patient experienced left laryngeal nerve palsy confirmed by laryngoscopy. Otherwise, there were no adverse events to include surgical-related mortality, recurrence of PDA, or need for chest tube placement during follow-up. SL of PDA in ELBW infants without chest tube placement is both efficient and safe. Universal consensus recommendations for the management of PDA in ELBW neonates are needed. Further study is required regarding the use of the less invasive option of percutaneous PDA closure in ELBW infants.

Abnormal origin of right pulmonary artery from the ascending aorta in an infant ("Hemitruncus").

Key Clinical Message: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital malformation and it needs surgical management. Consequences of this condition affect lead to pulmonary hypertension and severe pulmonary vascular disease. Abstract: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.

Investigation of the Role of BMP2 and -4 in ASD, VSD and Complex Congenital Heart Disease.

Congenital heart malformations (CHMs) make up between 2 and 3% of annual human births. Bone morphogenetic proteins (BMPs) signalling is required for chamber myocardium development. We examined for possible molecular defects in the bone morphogenetic protein 2 and 4 (BMP2, -4) genes by sequencing analysis of all coding exons, as well as possible transcription or protein expression deregulation by real-time PCR and ELISA, respectively, in 52 heart biopsies with congenital malformations (atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy ofFallot (ToF) and complex cases) compared to 10 non-congenital heart disease (CHD) hearts. No loss of function mutations was found; only synonymous single nucleotide polymorphisms (SNPs) in the BMP2 and BMP4 genes were found. Deregulation of the mRNA expression and co-expression profile of the two genes (BMP2/BMP4) was observed in the affected compared to the normal hearts. BMP2 and -4 protein expression levels were similar in normal and affected hearts. This is the first study assessing the role of BMP-2 and 4 in congenital heart malformations. Our analysis did not reveal molecular defects in the BMP2 and -4 genes that could support a causal relationship with the congenital defects present in our patients. Importantly, sustained mRNA and protein expression of BMP2 and -4 in CHD cases compared to controls indicates possible temporal epigenetic, microRNA or post-transcriptional regulation mechanisms governing the initial stages of cardiac malformation.

Outcomes after repair of complete atrioventricular canal with a modified single-patch technique: a retrospective study.

BACKGROUND: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. METHODS: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. RESULTS: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). CONCLUSION: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

Converting the Fontan Circulation: Challenges and Evolution.

The Fontan operation, the final palliative step after a series of complex operations in patients with univentricular hearts, has undergone multiple modifications throughout the last decades, with the goal of finding the method which combines the optimal hemodynamic effects of the Fontan circulation with minimal long term side effects. An understanding of the operative evolution and subsequent side effects, as well as the management thereof seems imperative. Since its inception by Francis Fontan the, now obsolete, initial atriopulmonary connection has passed through several milestones into having now reached the era of total cavopulmonary connection. However, recently published results bring to light the new option of biventricular conversion which comes to challenge the management of Fontan patients as we know it. Currently, there is no consensus as to whether to continue with palliation in this challenging group of patients or proceed with heart transplantation. In this collective review, we provide a historic overview of the Fontan evolution as well as future insights, discussing the perspectives of options for patients with failing Fontan, including the latest addition of biventricular correction.

Acute Kidney Injury Predictors and Outcomes after Cardiac Surgery in Children with Congenital Heart Disease: An Observational Cohort Study.

Acute Kidney Injury (AKI) commonly complicates cardiac surgery in children with congenital heart disease (CHD). In this study we assessed incidence, risk factors, and outcomes of postoperative AKI, while testing the hypothesis that, depending on the underlying diagnosis, there would be significant differences in AKI incidence among different diagnostic groups. We conducted an observational cohort study of children with CHD undergoing cardiac surgery in a single tertiary center between January 2019 and August 2021 (n = 362). Kidney Disease Improving Global Outcome (KDIGO) criteria were used to determine the incidence of postoperative AKI. Diagnosis was incorporated into multivariate models using an anatomic-based CHD classification system. Overall survival was estimated using Kaplan−Meier curves. Log-rank test and adjusted Cox proportional hazard modelling were used to test for differences in survival distributions and determine AKI effect on survival function, respectively. AKI occurred in 70 (19.3%), with 21.4% in-hospital mortality for AKI group. Younger age, lower weight, longer cardiopulmonary bypass time, preoperative mechanical ventilation and diagnostic category were associated with postoperative AKI. Resolution rate was 92.7% prior to hospital discharge for survivors. AKI was associated with longer duration of mechanical ventilation, ICU and hospital length of stay. AKI patients had significantly higher probability of all-cause mortality postoperatively when compared to the non-AKI group (log-rank test, p < 0.001). Adjusted hazard ratio for AKI versus non-AKI group was 11.08 (95% CI 2.45−50.01; p = 0.002). Diagnostic category was associated with cardiac surgery-related AKI in children with CHD, a finding supporting the development of lesion specific models for risk stratification. Postoperative AKI had detrimental impact on clinical outcomes and was associated with decreased survival to hospital discharge.

Intraoperative diagnosis of retroaortic left innominate vein in a patient with congenital heart disease.

Diagnosis of retroaortic left innominate vein is usually made by echocardiography, computed tomography, and magnetic resonance imaging, but in several cases, diagnosis is made in the theater.

Congenital, acquired, or both? The only two congenitally based, acquired heart diseases.

Discrete subaortic stenosis (DSS) is a type of left ventricular outflow tract obstruction whereas double-chambered right ventricle is a form of right ventricular outflow tract obstruction. Both of these cardiac malformations share lots of similar characteristics which classify them as acquired developmental heart diseases despite their congenital anatomical substrate. Both of them are frequently associated to ventricular septal defects. The initial stimulus in their pathogenetic process is anatomical abnormalities or variations. Subsequently, a hemodynamic process is triggered finally leading to an abnormal subaortic fibroproliferative process with regard to DSS or to hypertrophy of ectopic muscles as far as double-chambered right ventricle is concerned. In many cases, these pathologies are developed secondarily to surgical management of other congenital or acquired heart defects. Moreover, high recurrence rates after initial successful surgical therapy, particularly regarding DSS, have been described. Finally, an interesting coexistence of DSS and double-chambered aortic ventricle has also been reported in some cases.

Right atrial thrombus mimicking cardiac tumor in a neonate.

Does the cardiac mass should be operated in neonates? When diagnosis of cardiac tumor or mass has been made, the surgical excision should not be delayed for prevention of life-treating complications.

Right superior vena cava draining into the left atrium in a patient with sinus venosus defect.

Right superior vena cava draining into the left atrium is an extremely rare anomaly of systemic venous return. It can be isolated or it can be associated with other congenital heart defects, thus clinical presentation may vary. A case of a 9-year-old asymptomatic patient with sinus venous defect and bilateral superior vena cava with the right-sided draining into the left atrium is described.

Surgical Management of an Aspergillus Empyema in a 3-Year-Old Child.

Aspergillus empyema in nonimmunocompromised children is rare. A case of surgical management of invasive aspergillosis in a previously healthy 3-year-old child is presented. The patient was initially admitted to a hospital with severe respiratory deterioration and clinical instability, originally attributed to sepsis. After surgical intervention and the diagnosis of invasive aspergillosis, intravenous therapy with voriconazole was initiated. During postoperative care, the patient's condition remained stable with mild functional respiratory deficits. The diagnosis and treatment of Aspergillus empyema remains challenging, especially in cases that the recognition of aspergillosis is delayed and urgent surgical management of the empyema is required due to rapid clinical deterioration of the patient. The early initiation, prolonged administration, and close monitoring of high-dose antifungal treatment are highly recommended.

An alternative technique for downsizing a modified Blalock-Taussig shunt.

An alternative surgical approach for downsizing an existed modified Blalock-Taussig shunt is described as a reoperation in a hemodynamically unstable patient. This method was selected in order to minimize the surgical manipulations in the setting of a critically ill infant.

An alternative technique to safely close the chest after placing a child on central venoarterial extracorporeal membrane oxygenation.

Central venoarterial (VA) placement of extracorporeal membrane oxygenation (ECMO) is performed surgically, and in the majority of cases, the patient remains with an open sternum. Herein, a case of a 3-year-old patient who underwent insertion of a central VA ECMO for heart failure due to acute myocarditis is described. An alternative technique for ECMO placement providing sternal closure and minimizing infection risk for the safe patient transport is described.

Left Upper Lobectomy for Congenital Lobar Emphysema in a Low Weight Infant.

Congenital lobar emphysema (CLE) is a rare lung congenital malformation. Differential diagnosis of the disease remains challenging in an infant with acute respiratory distress. We report a case of a 3-week-old female infant with a weight of 2.1 kg who presented respiratory distress related to CLE. Left upper lobectomy was performed and she had an uneventful recovery.

One-Stage Repair of an Interrupted Aortic Arch with an Aortopulmonary Window in a Premature Neonate.

Interrupted aortic arch with an aortopulmonary window is a rare congenital entity that is associated with high morbidity and mortality, especially in premature low-birth-weight infants, and the proper timing of surgical correction remains a matter of debate. We present the case of a premature infant weighing 1.6 kg who successfully underwent one stage surgical repair to treat interrupted aortic arch with an aortopulmonary window. The therapeutic management of this patient is described below, and a review of the literature is presented.

Etiology and management of chylothorax following pediatric heart surgery.

BACKGROUND: Chylothorax after congenital heart surgery (CHD) is a potentially challenging complication. The purpose of this study was to review our experience with the management of chylothorax following congenital heart surgery. METHODS: Between September 1997 and August 2006, of 1341 pediatric patients undergoing correction of congenital heart disease in our institution, 18 (1.3%) developed chylothorax postoperatively. Surgical procedures included tetralogy of Fallot repair in 10 patients, ventricular septal defect closure (one), atrial septal defect with pulmonary stenosis repair (one), Fontan procedure (three), coarctation of the aorta repair (one), aortopulmonary shunt (one), and ligation of patent ductus arteriosus in one patient. All patients followed a therapeutic protocol including complete drainage of chyle collection and controlled nutrition. Somatostatin was used adjunctively in six (33.3%) patients. Surgical intervention was reserved for persistent lymph leak despite maximal therapy. Following resolution of chylothorax, a medium-chain triglyceride diet was implemented for six weeks. RESULTS: There were no deaths. Fifteen patients (83.3%) responded to conservative therapy. Lymph leak ranged from 2.5 to 14.7 mL/kg per day for 8 to 42 days. Three patients with persistent drainage required thoracotomy with pleurodesis to achieve resolution, in two of which previously attempted chemical pleurodesis with doxycycline proved ineffective. Duration of lymph leak in this subgroup ranged from 15 to 47 days with 5.1 to 7.4 mL/kg per day output. CONCLUSIONS: Postoperative chylothorax is an infrequent complication of surgery for congenital heart disease and can occur even after median sternotomy in the absence of pathologically elevated venous pressure or Fontan circulation. Although hospitalization can be prolonged, conservative therapy is effective in most cases, while surgical pleurodesis proved successful in the refractory cases.

Successful surgical repair of a giant arteriovenous fistula of the coronary arteries.

Large coronary arteriovenous fistulas (CAVF) cause severe symptoms and their repair involves the serious challenge of occluding the fistula while simultaneously preserving normal coronary flow. We present a case of a giant CAVF involving the right and left coronary arteries in a symptomatic adult patient and its successful surgical repair.

Surgical results after total transatrial/transpulmonary correction of tetralogy of Fallot.

INTRODUCTION: Surgical repair of tetralogy of Fallot is associated with low early morbidity and mortality. However, there may be late morbidity and mortality due to right ventricular dysfunction. The transatrial/transpulmonary technique may ameliorate these long-term complications. Here we present the results from our use of this approach. METHODS: A hundred sixty-three consecutive patients (age 6 months to 45 years, median 1.5 years) underwent transatrial/transpulmonary total correction in our department. In 142 patients the main pulmonary artery was augmented by an autologous pericardial patch, in 31 cases the arterioplasty was extended to the pulmonary artery branches, and pulmonary artery valvuloplasty was needed in 129 patients. A monocusp autologous pericardial valve mechanism was inserted in 14 patients. RESULTS: Patient follow up was 100% complete with a median duration of 3.05 years. There were no deaths. One patient required early reoperation to relieve residual right ventricular outflow tract (RVOT) obstruction. Median ICU and hospital stay were 3 and 11 days, respectively. At hospital discharge RVOT gradient was 13.7 +/- 13 (median 10) mmHg, while most patients (94%) had up to moderate pulmonary valve insufficiency (1 + in 63.8%, 2+ in 30.6%), and normal (92.6%) or mildly reduced (6.1%) right ventricular function. In 81% some degree of tricuspid regurgitation was noted. One patient required late reoperation for mitral valve repair. All patients are in NYHA class I or II. The degree of pulmonary valve insufficiency remains stable (69.9% with 0-1 + and 24.5% up to 2+). Likewise, tricuspid valve function remains unchanged (96% of the patients had mild or up to moderate regurgitation). There was no significant RVOT obstruction and in most patients (93.2%) right ventricular function was normal. CONCLUSION: These results compare very favorably to those reported in the literature. The medium-term findings auger well for future adverse event rates, but long-term follow up is still necessary to confirm them.