The Fate of Mitral Valve Surgery in the Pediatric Age: A 25-Year Single-Center Experience.

Background: The aim of this study was to evaluate the natural history of patients after mitral valve intervention in the pediatric age. Methods: This is a retrospective study including all patients who underwent mitral valve surgery from 1998 to 2022. The patients' surgical reports, postoperative records, and ambulatory visits were reviewed. The endpoints of the study were survival and freedom from mitral valve reoperation. Results: Of the 70 patients included in the cohort, 61 patients (86.7%) had congenital mitral valve disease, of whom 46 patients (75.4%) had a predominantly mitral regurgitation lesion, and 15 patients (24.6%) had a predominantly mitral stenosis. In the mitral regurgitation group, all of the patients underwent valve repair with an operative mortality of one patient (2.1%), and with median follow-up of 4 years (range, 0.5-13 years), there was 4.3% mortality (n = 2) and 71.2% freedom from reoperation. In the mitral stenosis group, 11 patients underwent mitral valve repair, and 4 patients underwent valve replacement. There was an operative mortality of two patients (13.3%). With a 2-year median follow-up (range: 0.1-23 years), there were no additional mortality cases in the mitral stenosis group. All three patients who survived primary mitral valve replacement (100%) and four patients who survived a primary repair (40.0%) underwent reoperation. Conclusions: This study demonstrates encouraging outcomes for mitral valve repair. The mortality of patients with congenital mitral valve disease may also be related to a difficult postoperative course, rather than the MV lesion itself.

The Impact of Dominant Ventricular Morphology on the Early Postoperative Course After the Glenn Procedure.

The dominant ventricular morphology affects both the early and late outcomes of the Fontan procedure, but its impact on the patients' status immediately following the Glenn procedure is unknown. This study aims to evaluate the effect of the infants' dominant ventricular morphology on the immediate course after undergoing the Glenn procedure. This single-center, retrospective study included all patients who underwent the Glenn procedure between October 2003 and May 2016. The patients were divided into two groups according to their dominant ventricular morphology. Their postoperative records were reviewed and compared. Out of the 89 patients who underwent the Glenn procedure during the study period, 40 (44.9%) had dominant right ventricular morphology and 49 (55.1%) had left ventricular morphology. There were no significant group differences in baseline characteristics or operative data. The maximal postoperative vasoactive-inotropic score was significantly higher and the extent of ventricular dysfunction was significantly more severe in the dominant right ventricle group (P < 0.05). The length of hospitalization was slightly but not significantly longer in the hypoplastic LV group. It is concluded that patients with a dominant LV morphology had a superior ventricular function and required less inotropic support compared to that of a dominant RV morphology in the immediate postoperative course following the Glenn procedure. Survival was not affected by these differences. Further study to determine the pathophysiologic basis for these differences is warranted.

Normothermic Versus Hypothermic Norwood Procedure.

BACKGROUND: Either deep hypothermia with circulatory arrest or hypothermic perfusion with antegrade selective cerebral perfusion is used during the Norwood procedure for hypoplastic left heart syndrome. Normothermic perfusion has been described for pediatric patients. The aim of this study was to compare the early outcomes of patients undergoing the Norwood procedure with antegrade selective cerebral perfusion under hypothermia with the procedure under normothermia. METHODS: From 2005 to 2020, 117 consecutive patients with hypoplastic left heart syndrome underwent the Norwood procedure: 68 (58.2%) under hypothermia and 49 (41.8%) under normothermia. Antegrade selective cerebral perfusion flow was adjusted to maintain right radial arterial pressure above 50 mm Hg, and a flow rate of 40 to 50 mL kg-1 min-1. Baseline characteristics, operative data, and postoperative outcomes including lactate recovery time were compared. RESULTS: The baseline characteristics and cardiovascular diagnosis were similar in both groups. The normothermic group had a significantly shorter bypass time (in minutes) of 90.31 (±31.60) versus 123.63 (±25.33), a cross-clamp time of 45.24 (±16.35) versus 81.93 (±16.34), and an antegrade selective cerebral perfusion time of 25.61 (±13.84) versus 47.30 (±14.35) (P < .001). There were no statistically significant differences in the immediate postoperative course, or in terms of in-hospital mortality, which totaled 9 (18.4%) in the normothermic group, and 10 (14.9%) in the hypothermic group (P = .81). CONCLUSION: The normothermic Norwood procedure with selective cerebral perfusion is feasible and safe in terms of in-hospital mortality and short-term outcomes. It is comparable to the standard hypothermic Norwood with selective cerebral perfusion.

Novel cfDNA Methylation Biomarkers Reveal Delayed Cardiac Cell Death after Open-heart Surgery.

The use of cardiopulmonary bypass (CPB) is thought to cause delayed cardiac damage. DNA methylation-based liquid biopsies are novel biomarkers for monitoring acute cardiac cell death. We assessed cell-free DNA molecules as markers for cardiac damage after open-heart surgery. Novel cardiomyocyte-specific DNA methylation markers were applied to measure cardiac cfDNA in the plasma of 42 infants who underwent open-heart surgery. Cardiac cfDNA was elevated following surgery, reflecting direct surgery-related tissue damage, and declined thereafter in most patients. The concentration of cardiac cfDNA post-surgery correlated with the duration of CPB and aortic cross clamping. Strikingly, cardiac cfDNA at 6 h predicted duration of mechanical ventilation and maximal vasoactive-inotropic score better than did maximal troponin levels. Cardiac cfDNA reveals heart damage associated with CPB, and can be used to monitor cardiac cell death, to predict clinical outcome of surgery and to assess performance of cardioprotective interventions.

The horizon of pediatric cardiac critical care.

Pediatric Cardiac Critical Care (PCCC) is a challenging discipline where decisions require a high degree of preparation and clinical expertise. In the modern era, outcomes of neonates and children with congenital heart defects have dramatically improved, largely by transformative technologies and an expanding collection of pharmacotherapies. Exponential advances in science and technology are occurring at a breathtaking rate, and applying these advances to the PCCC patient is essential to further advancing the science and practice of the field. In this article, we identified and elaborate on seven key elements within the PCCC that will pave the way for the future.

Dominant Ventricular Morphology and Early Postoperative Course After the Fontan Procedure.

BACKGROUND: Single ventricle heart disease comprises a wide variety of critical heart defects that lead to the provision of systemic cardiac output by one dominant ventricle. It requires staged surgical palliation that culminates in Fontan circulation. Dominant ventricular morphology in single ventricle patients reportedly has an impact on postoperative morbidity and mortality with varying results. The objectives of this study were to examine the association between ventricular morphology and the early postoperative course after the Fontan procedure. METHODS: A retrospective cohort study in a tertiary referral pediatric medical center that included 98 consecutive patients who underwent Fontan procedure between October 2009 and May 2016. Postoperative outcomes were compared between patients with left ventricular morphology and those with right ventricular morphology (crude effect and regression analysis). RESULTS: Patients with right ventricular morphology had longer postoperative hospitalizations compared to patients with left ventricular morphology (26.5 days vs 18.2 days, respectively, P = .028), higher postoperative maximal vasoactive-inotropic scores (25.6 vs 12.4, P = .02), higher serum lactate levels (7.7 mmol/L vs 6.4 mmol/L, P = .03), higher proportions of ventilation throughout 24 h or more (16 patients [38%] vs 8 patients [14%], P = .009), higher proportions of ventricular dysfunction (12 patients [29%] vs 5 patients [9%], P = .0001), and lower blood oxygen saturation levels at discharge (87% vs 92%, P = .03). CONCLUSIONS: The Fontan procedure in patients with right ventricular morphology is associated with longer postoperative hospitalization and worse early postoperative characteristics (ventricular dysfunction and atrioventricular valve regurgitation) as well as higher rates of early, transient signs of sub-optimal postoperative hemodynamics compared to those with left ventricular morphology.

Catheter-directed thrombolysis for in situ pulmonary artery thrombosis in children.

In situ pulmonary artery thrombosis (ISPAT) is a unique form of pulmonary embolism characterized by local formation of thrombus in the pulmonary arteries. We present here a baby with hypoplastic left heart syndrome who developed ISPAT after Glenn surgery. The patient underwent catheter-directed thrombolysis, followed by systemic anticoagulation with excellent results.

Transfusion-related acute hepatic injury following postoperative platelets administration in pediatric patients undergoing the Fontan procedure.

OBJECTIVE: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury. DESIGN: Single center retrospective cohort study. SETTING: Pediatric Cardiac Intensive Care Unit at a tertiary medical center. PATIENTS: Ninety-nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Out of the four types of blood products, transfusion of platelets was found to significantly affect postoperative levels of ALT and AST. Additional factors included postoperative administration of sodium bicarbonate, decreased flow through the Fontan canal and decreased urine output. Preoperative pulmonary artery pressure and pulmonary vascular resistance, cardiopulmonary bypass time, aortic cross-clamp time, amount of postoperative bleeding, and vasoactive-inotropic score did not influence liver enzymes levels CONCLUSIONS: In pediatric Fontan patients, platelets transfusions contribute to an acute hepatic injury. The relation between platelets and transfusion-related acute lung injury (TRALI) has been well described, but this is the first time it is being described in regard to acute hepatic injury (TRAHI). Changing platelet transfusion strategy could decrease morbidity in Fontan patients but further research is needed.

Dehydration as a Rare Cause of Pulmonary Artery Thrombosis in a 2-Week-Old Term Neonate.

Pulmonary arterial thrombosis is an extremely rare occurrence in the neonatal population. We describe a 2-week-old female neonate who presented in critical condition with severe cyanosis and dehydration and was found to have a large thrombus in the main branches of the pulmonary arteries. She was successfully treated with surgical embolectomy. Pulmonary arterial thrombosis should always be considered in the differential diagnosis of a dehydrated neonate presenting with severe cyanosis and evidence of pulmonary hypertension.

Heparin-induced thrombocytopenia complicating children after the Fontan procedure: Single-center experience and review of the literature.

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. The risk for HIT correlates with the cumulative dosage of heparin exposure. In Fontan patients, recurrent systemic anticoagulation, traditionally with heparin, is used to alleviate the thrombotic complications that may occur postoperatively when the venous pressure rises and the systemic venous flow into the pulmonary arteries becomes sluggish, putting them at increased risk. As a pressure gradient-dependent circulation, elevation in systemic venous pressure, most often by venous thrombosis, contributes to circuit failure. Therefore, when HIT complicates patients after the Fontan procedure, it is associated with a high thrombotic morbidity and mortality; thus, a high index of suspicion is mandatory, based on the clinical signs of HIT. It is crucial to intervene early with alternative anticoagulants when HIT is suspected as this step may improve outcome in these patients.

Prolonged prostaglandin E1 therapy in a neonate with pulmonary atresia and ventricular septal defect and the development of antral foveolar hyperplasia and hypertrophic pyloric stenosis.

Prostaglandin E1 (alprostadil) is widely used for maintaining the patency of ductus arteriosus in ductus-dependent congenital heart defects in neonates to improve oxygenation. Among more common side effects are fever, rash, apnoea, diarrhoea, jitteriness, and flushing. More severe side effects are brown fat necrosis, cortical hyperostosis, and gastric outlet obstruction, most commonly the result of antral foveolar hyperplasia or hypertrophic pyloric stenosis. We report on an infant with a ductus-dependent congenital heart defect who developed symptoms and sonographic evidence of focal foveolar hyperplasia and hypertrophic pyloric stenosis after prolonged treatment with prostaglandin E1. Gastrointestinal symptoms persisted after corrective cardiac surgery, and pyloromyotomy was required. Study of the case and of available literature showed an association between the total dose of prostaglandin E1 administered and duration of treatment and the development of gastric outlet obstruction. We conclude that if patients are treated with a prostaglandin E1 infusion, careful monitoring for symptoms and signs of gastric outlet obstruction is required.

Point of care testing in children undergoing cardiopulmonary bypass.

BACKGROUND: Excessive hemorrhage is a major complication after cardiac surgery requiring cardiopulmonary bypass (CPB). The aim of this study was to define the potential role of the cone and platelet analyzer (CPA) and the rotating thromboelastogram (ROTEM) point of care tests in children undergoing CPB. PROCEDURE: We prospectively studied 15 pediatric patients aged 1 month to 10 years. Blood count, blood coagulation tests (prothrombin time [PT], activated partial thromboplastin time, fibrinogen, thrombin time), CPA and ROTEM parameters were measured before and after CPB. Demographic and surgical data were recorded as were those on perioperative blood loss and blood product transfusion. RESULTS: Low body weight, longer duration on CPB and lower core body temperature were associated with an increased bleeding risk. The ROTEM test showed a significant prolongation of clotting time and decreased maximal clot firmness (MCF) postoperatively in children with increased bleeding. The coagulation parameters associated with increased bleeding were: prolonged PT, lower fibrinogen levels, prior to surgery, and lower MCF after surgery. CPA test findings were not associated with postoperative bleeding in our patients. CONCLUSIONS: CPA did not serve as a prognostic tool for predicting bleeding risk in children undergoing CPB. The change in ROTEM's post-CPB results associated with bleeding tendency, and they may predict for poorer clot formation and stability.

A randomized trial of outcomes of anesthetic management directed to very early extubation after cardiac surgery in children.

OBJECTIVES: Intraoperative management directed to early extubation of children undergoing cardiac surgery has been suggested as a viable alternative to prolonged postoperative mechanical ventilation. The authors evaluated the safety and efficacy of this approach in a randomized prospective trial. DESIGN: A prospective randomized observational study. SETTING: A single university-affiliated hospital. PARTICIPANTS: One hundred consecutive pediatric patients (age 1 month-15 years, weight 3.0-51 kg) requiring cardiac surgery. Patients younger than 1 month of age and those requiring mechanical ventilation before the operation were considered ineligible for the study. INTERVENTIONS: Patients were randomly allocated to a group with anesthetic management and extubation in the operating room (early group [EG]) and a group with elective prolonged mechanical ventilation (control group [CG]). MEASUREMENTS AND MAIN RESULTS: A difference in outcome as reflected by the pediatric intensive care unit (PICU) and hospital lengths of stay and postoperative morbidity and mortality was analyzed. A separate analysis was performed in children younger than 3 years old. The extubation time in the CG was 25.0 +/- 26.9 hours. No differences in mortality, the need for re-exploration for bleeding, the need for reintubation, the incidence of abnormal chest radiographic findings, or cardiac and septic complications between groups were found. PICU and postoperative hospital lengths of stay were significantly shorter in patients in the EG (3.3 +/- 1.9 days in the EG v 5.8 +/- 4.1 in the CG, p < 0.001, and 7.4 +/- 2.9 days in the EG v 11.2 +/- 6.8 days in the CG, p = 0.009). CONCLUSIONS: In children undergoing cardiac surgery, anesthetic management with early cessation of mechanical ventilation appears to be safe and decreases hospital and PICU length of stay. However, because the size of the study did not allow for the detection of possible differences in perioperative mortality, only a large multicenter study may provide a definite answer to this question. The present study may be treated as a pilot for such a trial.

Accessory mitral valve tissue: a case report and an updated review of literature.

Accessory mitral valve tissue is an unusual congenital cardiac anomaly and a rare cause responsible for left ventricular outflow tract obstruction. An 18-year-old patient was referred to this hospital due to an occasionally noted heart murmur in a medical examination. Echocardiography facilitated the diagnosis of accessory mitral valve tissue. To relieve the left ventricular outflow tract obstruction, an operation including resection of the accessory mitral valve tissue, implantation of artificial chordae tendineae, and mitral valve annuloplasty was performed successfully. Postoperative echocardiography showed a complete relief of the mitral valve leaflets and a wide patent left ventricular outflow tract. However, transient ischemic attack and Horner's syndrome complicated the patient early postoperatively. He was administered with a high dose of aspirin, and he recovered shortly. Surgical removal is in so much mandatory as a definite diagnosis of accessory mitral valve tissue with left ventricular outflow tract obstruction is established. A prophylactic treatment should be applied to the patients with accessory mitral valve tissue in virtue of their susceptibility to neurological events.

An incomplete pentalogy of Cantrell.

Pentalogy of Cantrell is a rare entity of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. The complete pentalogy and its variants have been described in the literature. We report a 47-day-old girl with an incomplete pentalogy of Cantrell presenting with congenital absence of the sternum, congenital heart defects, and an epigastric hernia. Two even rarer expressions of this disorder, tricuspid atresia type IIc and asymmetric kidneys, were also found. The patient was successfully managed with palliative pulmonary artery banding as preparation for a modified Fontan operation when she is older. We report this rare case, and discuss the pathologic findings and surgical strategy.

Right ventricular outflow tract reconstruction: valved conduit of choice and clinical outcomes.

BACKGROUND: The valved conduit of choice in right ventricular outflow tract (RVOT) reconstruction provides a challenge for cardiac surgeons. The present study collected data regarding the clinical outcome of valved conduits for RVOT reconstruction, so as to explore various options of ideal conduits in clinical practice. METHODS: English language articles on valved conduits for RVOT reconstruction were retrieved from the MEDLINE database with respect to the commonly used homograft, stented xenograft and stentless xenograft, and the occasionally used autologous tissue valved conduit as well. Clinical outcomes of each conduit were outlined with respect to their early and late mortalities, conduit failure, conduit reoperation, reoperation-free interval, actuarial freedom from reoperation, and survival rates. Conduit-related complications, risk factors and pathological findings of the valved conduits were summarized. RESULTS: Conduit failure was defined as the need for reoperation for conduit stenosis or extrinsic compression, conduit regurgitation, or anastomotic dehiscence. The conduit failure rates at 2 years were 9-55%, 35% and 25% for homograft, stented xenograft and stentless xenograft conduits, respectively. The 5-year actuarial freedoms from reoperation were 87-98.2% for homograft, 37% for Hancock, 81-92% for Carpentier-Edwards, 78% for Contegra, and 82.95% for LabCor, respectively. The result for Hancock at 5 years appeared to be disappointing, although it did prove promising, and was 79.5% at 10 years and 65.8% at 15 years. Autologous pericardial valved conduits for RVOT reconstruction showed superb properties, and the autologous monocusp pulmonary artery conduit functioned well early postoperatively, but data for long-term follow-up are lacking. CONCLUSION: Conduit failure and explant is inevitable. This phenomenon is worse with a longer follow-up. Mechanisms involved in conduit failure are unknown, even though they were accounted for by calcification and extensive intimal proliferation, and somatic outgrowth. Homografts are commonly used and have experienced a long history. The pulmonary homograft is the most commonly used RVOT conduit, especially in small children, due to its excellent characteristics. The newly-developed Contegra conduit has become popular due to its availability in full sizes and the acceptable results obtained at intermediate follow-up. The Hancock conduit can function sufficiently well for as long as 5-10 years, and early valve failure is relatively rare. It is admissible to use the Hancock conduit as an interim measure for future conduit reoperation due to its adequate function until subsequent operation. The application of an autologeous tissue valved conduit should be considered when other alternatives are not available.

Minimally invasive congenital cardiac surgery through right anterior minithoracotomy approach.

BACKGROUND: Median sternotomy has been the conventional approach for correction of congenital cardiac defects despite poor cosmetic results at times. Right anterior minithoracotomy was, therefore, assessed as an alternative procedure with a better cosmetic outcome. METHODS: From October 2002 through February 2007, 75 patients underwent correction of congenital cardiac malformations with the use of cardiopulmonary bypass through right anterior minithoracotomy involving a short incision through the fifth intercostal space and the minimally invasive cannulation. Of them, 18 patients were infants, 42 were children, and 15 were adult. The average age was 9.26 +/- 14.1 years (range, 1.2 to 56). The average weight was 19.59 +/- 24.3 kg (range, 8.5 to 118 kg). The corrected defects included atrial septal defect type II, sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage, atrial component of atrioventricular septal defect, perimembranous ventricular septal defects with patent foramen ovale, mitral valve repair (complex), repair of cleft mitral valve, cor triatum atrial septal defect, repair of double-chambered right ventricle and extraction of atrial septal defect closure device. Skin incisions were as long as 5 cm. RESULTS: There was no operative or late mortality or major morbidity. The mean cardiopulmonary bypass time was 58.67 +/- 35.11 minutes (range, 32 to 263). Sixty-five patients were extubated in the operating room; the remaining 10 patients were extubated within 4 hours. Cosmetic result was very satisfactory in all patients. Two adult patients complained of some right chest musculoskeletal discomfort. CONCLUSIONS: The right anterior minithoracotomy incision is a safe and effective alternative to a median sternotomy for correction of congenital heart defects. Cosmetic results are highly satisfactory.

Mitral valve destruction by Hodgkin's lymphoma-associated Loefler endocarditis.

We present a case of nearly total posterior mitral leaflet destruction due to Loeffler endocarditis associated with Hodgkin's lymphoma in a 15-year-old adolescent. The patient was treated medically with subsequent surgical mitral valve replacement. Cardiac aspects of hypereosinophilic syndrome are also discussed.

The role of flexible fibreoptic bronchoscopy in evaluation of pulmonary diseases in children with congenital cardiac disease.

BACKGROUND: Diseases of the lungs and airways can be life-threatening in patients with congenital cardiac disease. External compression, and tracheobronchomalacia, as well as pathology of the large airways, can cause respiratory symptoms such as wheezing, stridor, fever, and dyspnoea, along with producing difficulty to wean the patient from mechanical ventilation. A definite diagnosis may be achieved by fibreoptic flexible bronchoscopy. AIMS: To characterize the patients who underwent bronchoscopy for respiratory diseases associated with congenital cardiac disease, and to evaluate the safety, total diagnostic rate, and diagnostic rate, which yielded alteration of treatment in this group of patients. METHODS: We reviewed retrospectively the records of 39 patients with congenital cardiac disease associated with abnormalities of the airways who underwent 49 fibreoptic bronchoscopies between 1999 and 2004. The patients were evaluated with regard to rate of diagnosis, the rate of procedures that resulted in alteration of management, and the rate of complications. RESULTS: Fibreoptic flexible bronchoscopy was safe, and contributed to a specific diagnosis in almost nine-tenths of the procedures. A specific diagnosis that resulted in treatment alteration was achieved in one-fifth of the procedures. CONCLUSIONS: Fibreoptic flexible bronchoscopy has an important role in the evaluation of pulmonary abnormalities in patients with congenital cardiac diseases, and should be considered as an initial diagnostic tool that may guide the clinician with regard to the proper therapy.

Site-specific delivery of dexamethasone from biodegradable implants reduces formation of pericardial adhesions in rabbits.

Repeated sternotomy often leads to serious complications in patients due to the formation of cardiac adhesions. In this study we characterized dexamethasone-loaded biodegradable poly(lactide)-poly(ethyleneglycol) copolymer films for site-specific drug delivery and examined their efficacy in the rabbit model of postoperative cardiac adhesions. Tritiated dexamethasone-loaded films were used to determine the in vitro release and in vivo drug distribution. Dexamethasone release in serum was biphasic with 69% drug released after 72 hr. The implants produced sustained drug levels at the implantation site with low distribution into the peripheral tissues. The matrices were implanted in rabbits between the epicardium and the sternum following sternotomy, pericardiectomy and epicardium abrasion, with the drug-releasing surface facing the epicardium. The tenacity and density of the adhesions was examined 21 days post procedure in comparison to both groups of untreated and rabbits implanted with blank matrices. Similarly tenacious and dense adhesions were observed in both control groups. In contrast, epicardial adhesions' formation was significantly reduced and the anatomy was preserved in the treated animals. It is concluded that local delivery of dexamethasone from biodegradable implants provides a promising approach for the prevention of pericardial adhesions while potentially minimizing the systemic adverse effects inherent to systemic therapy or high blood levels of the drug.