Short- and long-term survival of children treated with ventricular assist devices in Spain, based on 15 years' experience.

OBJECTIVES: To describe the use of ventricular assist devices (VAD) in children in Spain and to identify variables related to survival. METHODS: This is an observational cohort study of all children younger than 18 years of age who underwent an initial implantation of a VAD at any of the 6 paediatric heart transplant centres from May 2006 to December 2020. Subjects were identified retrospectively from each hospital's database. RESULTS: Paracorporeal VADs were implanted in 118 children [pulsatile (63%), continuous (30.5%) or both types (5.9%)]. Small children (<0.7 m2 of body surface area) comprised the majority of this cohort (63.3%). Overall, 67% survived to VAD explantation, and 64.9% survived to hospital discharge. Non-central nervous system haemorrhage (39%) and stroke (38.1%) were the most common complications. Body weight <5 kg, congenital heart disease, pre-implantation bilirubin >34 µmol/l and bridge to decision strategy were associated with a higher mortality at hospital discharge and in the long-term. Interagency registry for mechanically assisted circulatory support (INTERMACS) status 1 and cardiac arrest prior to VAD implantation were related to long-term mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not related to mortality. CONCLUSIONS: In Spain, 67% of the VAD-supported children have been bridged to heart transplantation or to recovery. Body weight lower than 5 kg, congenital heart disease diagnosis, cholestatic liver dysfunction, bridge to decision as VAD strategy, INTERMACS-1 status and cardiac arrest were pre-implantation variables related to mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not.

Differences between genetic dilated cardiomyopathy and myocarditis in children presenting with severe cardiac dysfunction.

Acute myocarditis is an inflammatory disease of the myocardium, and it can present as severe heart failure in children. Differential diagnosis with genetic cardiomyopathy can be difficult. The objective of this study is to identify patterns of clinical presentation and to assess invasive and non-invasive measures to differentiate patients with acute myocarditis from patients with dilated genetic cardiomyopathy. We performed a retrospective descriptive study of all paediatric patients (0-16 years old) that presented with new-onset heart failure with left ventricle ejection fraction < 35% in whom we performed an endomyocardial biopsy (EMB) during the period from April 2007 to December 2020. The patients were classified into two groups: Group 1 included 18 patients with myocarditis. Group 2 included 9 patients with genetic cardiomyopathy. Findings favouring a diagnosis of myocarditis included a fulminant or acute presentation (77.8% vs 33.3%, p = 0.01), higher degree of cardiac enzyme elevation (p = 0.011), lower left ventricular dimension z-score (2.2 vs 5.4, p = 0.03) increase of ventricular wall thickness (88.8% vs 33.3%, p = 0.03) and oedema in the EMB. Seven (77.8%) patients with genetic cardiomyopathy had inflammation in the endomyocardial biopsy fulfilling the diagnostic criteria of inflammatory cardiomyopathy.Conclusion: Differentiating patients with a myocarditis from those with genetic cardiomyopathy can be challenging, even performing an EMB. Some patients with genetic cardiomyopathy fulfil the diagnostic criteria of inflammatory cardiomyopathy. Using invasive and non-invasive measures may be useful to develop a predictive model to differentiate myocarditis from genetic cardiomyopathy. What is Known: • Acute myocarditis could present with cardiogenic shock in paediatric patients. • Parvovirus B19 is the main cause of myocarditis in this population. What is New: • Current diagnostic criteria for myocarditis have limited use in paediatric patients presenting with new-onset heart failure. • Some patients with a genetic cardiomyopathy and a new-onset heart failure fulfill the diagnostic criteria of inflammatory cardiomyopathy.

Usefulness of transaortic approach for a complex double-outlet right ventricle.

Surgical procedures for double-outlet right ventricle with ventricular septal defect are based on rerouting the blood flow of the left ventricle to the aorta through the ventricular septal defect (VSD) with an intraventricular baffle. The right atriotomy is the most common approach combined with a right ventriculotomy in some cases, particularly in pulmonary stenosis association. However, in complex cases, this standard operative strategy may not provide an adequate exposure. We describe the transaortic approach as an alternative procedure to repair a complex case of double-outlet right ventricle (DORV) with subaortic stenosis. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-021-01261-7.

Transient upper limb ischaemia during veno-arterial extracorporeal membrane oxygenation in a child.

Neck cannulation is the most common cannulation strategy performed to provide veno-arterial extracorporeal membrane oxygenation support in paediatric patients, especially in small children. Upper limb ischaemia is a rare complication of neck cannulation and is likely caused by arterial cannula malposition. We describe a case of right arm ischaemia caused by extrinsic compression of the right subclavian artery by the venous drainage cannula inserted through the right internal jugular vein. Upper limb hypoperfusion was resolved immediately after changing the venous drainage cannula from the right jugular vein to the right femoral vein.

Antenatal and Perioperative Mechanisms of Global Neurological Injury in Congenital Heart Disease.

Congenital heart defects (CHD) is one of the most common types of birth defects. Thanks to advances in surgical techniques and intensive care, the majority of children with severe forms of CHD survive into adulthood. However, this increase in survival comes with a cost. CHD survivors have neurological functioning at the bottom of the normal range. A large spectrum of central nervous system dysmaturation leads to the deficits seen in critical CHD. The heart develops early during gestation, and CHD has a profound effect on fetal brain development for the remainder of gestation. Term infants with critical CHD are born with an immature brain, which is highly susceptible to hypoxic-ischemic injuries. Perioperative blood flow disturbances due to the CHD and the use of cardiopulmonary bypass or circulatory arrest during surgery cause additional neurological injuries. Innate patient factors, such as genetic syndromes and preterm birth, and postoperative complications play a larger role in neurological injury than perioperative factors. Strategies to reduce the disability burden in critical CHD survivors are urgently needed.

Pediatric Tricuspid Valve Replacement With Transcatheter Bioprosthetic Valve: An Alternative Option in High-Risk Patients.

Transcatheter valve can be an alternative option of pediatric valve replacement in high-risk patients. We present 2 cases of Edwards Sapien 3 implantation in tricuspid position.

Myocardial protection in the arterial switch operation: Custodiol versus cold blood cardioplegia.

OBJECTIVES: The optimal myocardial protective solution in the neonatal arterial switch operation remains controversial. The aim of this study was to demonstrate that Bretschneider's histidine-tryptophan-ketoglutarate crystalloid solution (Custodiol) offers protection at least similar to that of cold blood cardioplegia. METHODS: Patients who underwent the neonatal arterial switch operation with Custodiol between January 2016 and December 2018 (n = 23) were compared with an historical cohort from August 2010 to December 2015 in which cold blood cardioplegia was used (n = 41). A linear mixed-effect model for repeated measures was performed to test the recovery of myocardial function based on inotropic and vasoactive inotropic scores, cardiac enzyme release and left ventricular ejection fraction. RESULTS: Patients in the cold blood cardioplegia group had higher inotropic scores in the first 24 h (0 h, P = 0.001 and 24 h, P = 0.006) and higher vasoactive inotropic scores in the first 72 h (0 h, 24 h and 48 h, P < 0.001; 72 h, P = 0.012). Cardiac troponin-I concentrations were higher in the cold blood cardioplegia group at postoperative hours 1-72 (1 h, 6 h, 12 h and 24 h, P < 0.001; 48 h, P = 0.001 and 72 h, P = 0.003). Creatinine-kinase-MB concentrations were higher in the cold blood cardioplegia group at postoperative hours 1-24 (1 h, 6 h and 12 h, P < 0.001; 24 h, P = 0.042). The left ventricular ejection fraction was higher in the Custodiol group just after the operation (P = 0.005), at 24 h (P = 0.001) and on the first day without inotropic support (P = 0.011). CONCLUSIONS: Neonatal myocardium protected with Custodiol during the arterial switch operation presented optimal ventricular function recovery with less inotropic support and less myocardial damage compared with cold blood cardioplegia.

A Venous Malformation: An Unusual Primary Cardiac Tumor in Children.

This case report describes a primary cardiac tumor, classified as venous malformation, diagnosed in an asymptomatic child. The tumor was located in the left atrium near the mitral valve without affecting the mitral valve's functioning. Complete resection of the lesion was performed because of the risk of systemic embolism. The lesion consisted of fibrous tissue with multiple venous vascular channels. The patient did not have similar lesions in other locations. Vascular primary cardiac tumors are extremely rare. Hemangiomas and lymphangiomas have been described previously, but to our knowledge, this is the first primary cardiac tumor identified as a venous malformation.

Prosthetic pulmonary valve and conduit endocarditis in congenital heart disease.

BACKGROUND: Pulmonary valve replacement is one of the most common procedures in patients with congenital heart disease. Little is known about prosthetic valve endocarditis in this population. OBJECTIVES: To review management and outcomes of pediatric and adult patients with a prosthetic pulmonary valve or right ventricle-to-pulmonary artery conduit infective endocarditis. METHODS AND RESULTS: A multiinstitutional cohort of 10 patients is reported. Median age at endocarditis was 24 years (range 8-41 years). The most common causative organisms were Staphylococcus ( n = 4) and Streptococcus species ( n = 2). In addition to medical treatment, 5 patients required surgery. Majors complications such as renal damage, life-threatening hemoptysis, and septic shock were observed. No patient died in hospital or during follow-up. CONCLUSIONS: Pulmonary prosthetic valve endocarditis is a rare condition associated with significant morbidity and a high risk of requiring operative intervention. Larger studies are required to optimize the management.

Late Dissection of a Contegra Conduit: A Rare Complication.

Surgical palliation of many types of congenital heart defects requires the use of a conduit between the right ventricle and the pulmonary artery. Dissections of these conduits are very infrequent. We report a case of a ten-year-old girl who developed acute right heart failure related to a Contegra conduit dissection. She underwent a new conduit replacement on an emergency basis.

Selective visceral perfusion improves renal flow and hepatic function in neonatal aortic arch repair.

OBJECTIVES: The aortic arch repair in the neonatal period is a complex procedure with significant morbidity. We define a useful double-perfusion technique and its effect on the function of abdominal organs in the postoperative course. METHODS: Nine patients with double perfusion (Group 1) were compared with 14 patients with antegrade cerebral perfusion (Group 2). The objective was to discern the incidence of postoperative acute kidney injury and impaired hepatic function, as well as tissue perfusion and myocardial function parameters. Mechanical ventilation time, postoperative length of stay and 30-day mortality were measured. We excluded patients with extracorporeal membrane oxygenation, early mortality (<72 h) and preoperative renal or hepatic insufficiency. RESULTS: Nine (39%) patients developed postoperative acute kidney injury, with 22% (n = 2) in Group 1 and 50% (n = 7) in Group 2 (P = 0.183). A higher urine output was observed during the first 24 h for Group 1 (P = 0.032). Eleven patients developed impaired hepatic function in the immediate postoperative period: 2 (18.2%) in Group 1 and 9 (81.8%) in Group 2 (P = 0.04). The international normalized ratio (P = 0.006-0.031) and prothrombin time (P = 0.007-P = 0.016) were significantly lower in the double-perfusion group during the first 72 h. Significant difference was observed in lactate levels in the first 72 h (P = 0.001-0.009). There was no postoperative mortality in either group. CONCLUSIONS: Selective visceral perfusion is a safe procedure that provides a better urine output, hepatic function and tissue perfusion. This technique allows for the repair of complex aortic arch anomalies in neonates without deep hypothermic circulatory arrest.

Caracterización y factores de riesgo de dilatación aórtica en pacientes pediátricos con válvula aórtica bicúspide / Characterization and risk factors for aortic dilatation in pediatric patients with bicuspid aortic valve

Introducción y objetivos: La dilatación de la aorta ascendente asociada a válvula aórtica bicúspide es una causa mayor de morbimortalidad en adultos. El objetivo principal fue reconocer la afectación aórtica en niños, así como sus características y factores de riesgo. Métodos: Se realizaron retrospectivamente las mediciones aórticas de todos los pacientes pediátricos con válvula aórtica bicúspide seguidos en un hospital pediátrico terciario entre 1997 y 2015. Se excluyeron los pacientes con síndromes asociados a dilatación aórtica (n=17). Resultados: Se incluyeron 206 pacientes, de los cuales el 67,9% eran varones. El patrón de apertura más común fue horizontal: 137 (66,7%). La mitad de los pacientes (101) tenía historia de coartación aórtica, 46 de ellos (22,3%) con estenosis aórtica≥moderada y 13 (6%) con insuficiencia aórtica≥moderada. El seguimiento medio fue de 6,1 (4,9) años; el diagnóstico de dilatación aórtica se realizó durante el primer año de seguimiento. La progresión de la dilatación de la aorta ascendente se documentó en el 17,1%, y en el 2,5% en la raíz aórtica. Más de un tercio (80/206) presentó dilatación aórtica (z-score>2). La afectación exclusiva de la aorta ascendente se produjo en 70/80 pacientes, con preservación de la raíz aórtica. En el análisis multivariado, los pacientes con dilatación de la aorta ascendente se asociaron a ausencia de coartación (p=0,001) y patrón de apertura vertical (p=0,007). Conclusiones: Está justificado el seguimiento en los pacientes pediátricos con válvula aórtica bicúspide debido a la frecuente asociación con disfunción valvular y/o dilatación aórtica (AU)

Introduction and objectives: Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors. Methods: Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17). Results: Two hundred and six patients were included, 67.9% males. The commonest opening pattern was horizontal: 137 (66.7%). Half of the patients (101) had a history of surgical aortic coarctation, 46 (22.3%) had≥moderate aortic valve stenosis and 13 (6%) had≥moderate aortic insufficiency. Mean follow-up time was 6.1 (4.9) years; diagnosis of aortic dilatation was made during the first year of follow-up. Progression of the dilatation of the ascending aorta was noted in 17.1%, and of the aortic root in 2.5%. More than one-third (80/206) had aortic dilatation (z-score>2). The ascending aorta was exclusively affected in 70/80 patients, with sparing of the aortic root. In the multivariate analysis, patients with dilatation of the ascending aorta were associated with absence of coarctation (P=.001) and vertical opening pattern (P=.007). Conclusions: Pediatric patients with bicuspid aortic valve warrant medical follow-up for the frequent association with valve impairment and/or dilatation of the ascending aorta (AU)

Characterization and risk factors for aortic dilatation in pediatric patients with bicuspid aortic valve. / Caracterización y factores de riesgo de dilatación aórtica en pacientes pediátricos con válvula aórtica bicúspide.

INTRODUCTION AND OBJECTIVES: Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors. METHODS: Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17). RESULTS: Two hundred and six patients were included, 67.9% males. The commonest opening pattern was horizontal: 137 (66.7%). Half of the patients (101) had a history of surgical aortic coarctation, 46 (22.3%) had≥moderate aortic valve stenosis and 13 (6%) had≥moderate aortic insufficiency. Mean follow-up time was 6.1 (4.9) years; diagnosis of aortic dilatation was made during the first year of follow-up. Progression of the dilatation of the ascending aorta was noted in 17.1%, and of the aortic root in 2.5%. More than one-third (80/206) had aortic dilatation (z-score>2). The ascending aorta was exclusively affected in 70/80 patients, with sparing of the aortic root. In the multivariate analysis, patients with dilatation of the ascending aorta were associated with absence of coarctation (P=.001) and vertical opening pattern (P=.007). CONCLUSIONS: Pediatric patients with bicuspid aortic valve warrant medical follow-up for the frequent association with valve impairment and/or dilatation of the ascending aorta.

Paediatric cardiac surgery in a peripheral European region: is a joint programme a safe alternative to regionalisation?

BACKGROUND: In 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands. METHODS: An operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated. RESULTS: In total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients). CONCLUSIONS: A joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

Bedside Ultrasound for the Diagnosis of Abnormal Diaphragmatic Motion in Children After Heart Surgery.

OBJECTIVE: To assess the utility of bedside ultrasound combining B- and M-mode in the diagnosis of abnormal diaphragmatic motion in children after heart surgery. DESIGN: Prospective post hoc blinded comparison of ultrasound performed by two different intensivists and fluoroscopy results with electromyography. SETTING: Tertiary university hospital. SUBJECTS: Children with suspected abnormal diaphragmatic motion after heart surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Abnormal diaphragmatic motion was suspected in 26 children. Electromyography confirmed the diagnosis in 20 of 24 children (83.3%). The overall occurrence rate of abnormal diaphragmatic motion during the study period was 7.5%. Median patient age was 5 months (range, 16 d to 14 yr). Sensitivity and specificity of chest ultrasound performed at the bedside by the two intensivists (91% and 92% and 92% and 95%, respectively) were higher than those obtained by fluoroscopy (87% and 83%). Interobserver agreement (k) between both intensivists was 0.957 (95% CI, 0.87-100). CONCLUSIONS: Chest ultrasound performed by intensivists is a valid tool for the diagnosis of diaphragmatic paralysis, presenting greater sensitivity and specificity than fluoroscopy. Chest ultrasound should be routinely used after pediatric heart surgery given its reliability, reproducibility, availability, and safety.