Timing of Pulmonary Valve Replacement: How Much Can the Right Ventricle Dilate Before it Looses Its Remodeling Potential?

Congenital heart disease patients that develop secondary pulmonary regurgitation require a pulmonary valve replacement (PVR) in their follow-up. The indications for PVR in asymptomatic patients are debated. Most guidelines consider a RV end-diastolic volume (RVEDV) over 150 ml/m(2) as an indication for PVR. We analyzed clinical, echocardiographic and MRI variables of patients that underwent a surgical PVR between September 2006 and February 2013. The included patients were asymptomatic, without pulmonary stenosis and with both pre- and post-surgery MRI. Thirty-five patients (74.3 % males) were included. Mean age at PVR was 25.8 years (SD = 7.18), and weight was 64.5 Kg (SD = 12.03). The main diagnosis was tetralogy of Fallot (n = 28), pulmonary atresia (n = 2), primary pulmonary regurgitation (n = 2) and pulmonary regurgitation after percutaneous treatment (n = 2). The maximal RVEDV pre-PVR was 267 ml/m(2), and right ventricular end-systolic volume (RVESV) was 183 ml/m(2). RV size and function were established by MRI: Pre-PVR Post-PVR p RVEDV (ml/m(2)) 162 (SD = 39.1) 94 (SD = 23.6) <0.001 RVESV (ml/m(2)) 87 (SD = 28.9) 44 (SD = 15.7) <0.001 RVEF 44.8 % (SD = 8.17) 52 % (SD = 9.9) <0.001 Patients with a RVEDV under 170 ml/m(2) combined with a RVESV under 90 ml/m(2) had a favorable RV remodeling, defined as RVEDV under 110 ml/m(2) (sensitivity 87.5 %), RVESV under 55 ml/m(2) (sensitivity 100 %) and RVEF over 50 % (sensitivity 100 %). When deciding the optimal PVR timing in asymptomatic patients, both RVEDV and RVESV should be considered. Our results suggest that higher volumes than used in the clinical practice can achieve a good remodeling. Therefore, PVR could be performed later in the follow-up reducing the number of cardiac interventions.

Infective endocarditis in congenital heart disease: a frequent community-acquired complication.

BACKGROUND: Infective endocarditis (IE) is a severe complication in patients with congenital heart disease (CHD). Epidemiology, etiology, and outcome in this group are different to those of patients with acquired heart disease. METHODS: We reviewed all cases of proven and probable IE (Duke's criteria) diagnosed in our center during the last two decades. RESULTS: We observed 45 cases of IE in patients with CHD (age range 8 months to 35 years); these represented 5.5 % of all the episodes of IE in our institution during the study period. The most frequent CHD were ventricular septal defect (31 %), tetralogy of Fallot (19 %), and atrioventricular septal defect (11 %). Twenty cases of IE (44 %) were recorded in patients with non-corrected native-valve CHD. Of the 24 patients with prosthetic-valve IE, post-operative acquisition during the first 6 months was confirmed in 11 patients (range 4-110 days). IE was community-acquired in 62 % of cases. Streptococcus spp. were the most frequent etiologic agents (33 %), followed by Staphylococcus spp. (32 %). Surgery was required to treat IE in 47 % of patients (52 % in prosthetic-valve IE and 41 % in native-valve IE, p = ns). In comparison to native-valve IE, prosthetic-valve IE was significantly more nosocomial-acquired (61 vs. 14 %, p = 0.002), presented a higher heart failure rate at diagnosis (39 vs. 9 %, p = 0.035), and developed more breakthrough bacteremia episodes (19 vs. 0 %, p = 0.048). Global mortality was 24 % (75 % in patients with prosthetic-valve IE who required surgery and 0 % in patients with native-valve IE who required surgery, p = 0.001). Multivariate analysis excluding breakthrough bacteremia (100 % mortality in this condition) confirmed that nosocomial IE [odds ratio (OR), 23.7; 95 % confidence interval (CI), 2.3-239.9] and the presence of heart failure at diagnosis of IE (OR, 25.9; 95 % CI, 2.5-269.6) were independent factors associated with mortality. CONCLUSION: Half of all cases of IE in patients with CHD occurred in patients with non-corrected native-valve CHD and two-thirds were community-acquired. Streptococcus spp. were the most frequent etiological agents. Patients with prosthetic-valve IE present a worse outcome, especially those requiring surgery. Breakthrough bacteremia, nosocomial IE, and heart failure are independent factors of mortality in patients with CHD presenting IE.

[Spanish multidisciplinary consensus on the prevention of respiratory syncytial virus infection in children]. / Consenso multidisciplinar español sobre la profilaxis de la infección respiratoria pediátrica por virus respiratorio sincitialConsenso.

BACKGROUND: A certain degree of feasibility exists in Spanish clinical practice with respect to interventions performed to prevent paediatric respiratory infection by RSV, including hygienic measures and intramuscular immunoprophylaxis with palivizumab. This task involves different paediatric specialties that may have a different perception of the magnitude of the problem and different professional criteria regarding the most appropriate actions. OBJECTIVES: To develop Spanish multidisciplinary consensus on preventing infection by RSV with the participation of the paediatric scientific societies involved (SNS, SSPC, SSPP and SSHPO). METHODS: Delphi Consensus modified in two rounds was used. The study was conducted in four phases: 1) constitution of a multidisciplinary Scientific Committee at the recommendation of the scientific entities participating in the study, for bibliographic review and submission of the recommendations to discussion; 2) constitution of an Expert Panel with 77 speciality representatives; 3) postal survey organised in two rounds and intermediate processing of opinions and issuing of a report for the panellists, and 4) discussion of the results in a meeting of the Scientific Committee. RESULTS: Consensus was reached on 48 of the 57 preventive recommendations analysed. With respect to the 9 remaining issues, no consensus was reached, due to differences in professional opinion and the absence of established criteria among the majority of the experts. Only in 3 recommendations was the opinion of the experts associated with the speciality of origin. CONCLUSIONS: A list of anti-RSV prophylactic recommendations was submitted, rated in accordance with the degree of professional consensus on which they were based. These can be considered valid until such time as new scientific information emerges that warrants a review thereof.

[Early postoperative complications after scoliosis surgery]. / Complicaciones médicas precoces en el postoperatorio de cirugía de escoliosis.

Several medical complications can occur after scoliosis surgery in children and adolescents. New surgical techniques have allowed greater degrees of scoliosis correction but have also increased the possibility of postsurgical deficit due to their greater aggressivity. We analyzed the early postsurgical complications of scoliosis surgery in a pediatric intensive care unit over a 10-year period. Seventy-six surgical procedures were performed. Of these, no complications occurred in 55 (73%). Chest X-ray revealed pulmonary atelectasia in 8 patients (10%) and pleural effusion in 7 patients (9%). Symptoms and signs of infection related to surgery were observed in only 6 children (8%). The absence of severe medical complications may be related to new surgical techniques and an experienced team.

[Early postoperative complications of thoracoscopy versus thoracotomy in pediatric idiopathic scoliosis]. / Estudio comparativo de las complicaciones postoperatorias inmediatas de la toracoscopia frente a la toracotomía en la escoliosis idiopática infantil.

INTRODUCTION: Thoracoscopic surgery has been used for anterior release, discectomy, and fusion in severe scoliosis or kyphosis. The indications of thoracoscopy for the treatment of pediatric spinal deformity are similar to those of thoracotomy-based spinal surgery. OBJECTIVE: We designed a comparative study to observe the immediate complications in the postoperative period after thoracoscopy or thoracotomy for scoliosis in the pediatric population. MATERIAL AND METHODS: The postsurgical complications of 63 interventions for idiopathic scoliosis over a 10-year period were analyzed. Conventional thoracotomy was used in 37 interventions (59 %) and thoracoscopy was used in 26 (41 %). RESULTS: In patients treated with the thoracolumbar endoscopic procedure, oral nutrition was resumed earlier, the mean length of hospital stay was lower, and debit drainage or requirement of surgical drainage were lower. CONCLUSIONS: Fewer immediate postsurgical complications were observed in scoliosis surgery when thoracoscopy was used.

[Echocardiographic study of hypertrophic myocardiopathy in children. Distribution and changing morphological course]. / Estudio ecocardiográfico de la miocardiopatía hipertrófica en niños. Distribución y evolución morfológica cambiante.

Thirty-eight children with diagnosis of hypertrophic cardiomyopathy were analyzed by M-Mode, two-dimensional and Doppler. The patients ranged in age from 7 days and 20 years (average 5 years). Three groups appear, based in morphologic myocardial affection. Patients with biventricular involvement ranged in age from 20 days and 6 months (group 1), patients aged until 6 years with left ventricular concentric morphology by echocardiographic examination (group 2), and the older in age group (group 3) with localized mid-ventricular hypertrophy in the same ventricle. A certain degree of obstruction was detected in 15 cases (46.8%). In both this aspect and systolic anterior motion of mitral valve and mitral regurgitation, the highest frequency was obtained in group 3. With respect to others echocardiographic parameters, like isovolumetric relaxation time and left ventricle short fraction, no variations have been observed between the three groups. Very interesting is the fact that one third of the patients of group 1 improved markedly, and another third of cases died. In conclusion, the echocardiography permits identify three groups in the hypertrophic cardiomyopathy in children, according to age and anatomic region affected. The variability of evolution in the youngest group of patients, suggest that certain cases are not true myocardiopathies, but myocardial hypertrophies secondaries to other problems. Only the oldest group supply the classics parameters of the adult hypertrophic cardiomyopathy.

[Contribution of color Doppler in the diagnosis of bilateral ductus arteriosus]. / Contribución del Doppler color al diagnóstico de ductus arterioso bilateral.

We report a neonate with distal bilateral ductus arteriosus origin of nonconfluent pulmonary arteries which was only diagnosed accurately by 2-D echo Doppler color flow mapping in the context of situs ambiguous, univentricular A-V connection and pulmonary atresia with normal systemic/pulmonary venous drainage. The color flow appearance of this singular vascular anatomy was enhanced after prostaglandin administration, which, allowed us to see an image resembling a "moustache morphology". At 36 months of age, and after a successful bilateral 4 mm modified Blalock Taussig shunt implantation by sequential approach, the patient is waiting for the next surgical step which will include a restoration of the confluence of the pulmonary arteries with bidirectional Glenn operation. Brief embryological and differential diagnosis comments are discussed.

[Practice Guidelines of the Spanish Society of Cardiology in the postoperative course of congenital heart diseases]. / Guías de práctica clínica de la Sociedad Española de Cardiología en el postoperado de cardiopatía congénita.

Improvements in myocardial protection, surgical techniques, and perioperative care have made it possible to achieve better prognosis in most congenital heart defects. This requires a coordinated, multidisciplinary approach to patient care, based on the preservation of adequate oxygen delivery to vital organs. It is important to have an understanding of normal postoperative status after cardiac surgery so that abnormal postoperative convalescence can be identified and treated.The causes of abnormal convalescence may be grouped into three categories: a) the pathophysiology of the defect before surgery and the acute changes in physiology that result from surgery; b) the effects of hypothermic cardiopulmonary bypass and deep hypothermic circulatory arrest on organ function, and c) the presence of residual anatomic defects. These conditions may result in prolonged convalescence as well as increased morbidity and mortality. Three primary hemodynamic pathophysiologic disturbances may occur during the postoperative period and lead to abnormal convalescence: left ventricular dysfunction, right ventricular dysfunction and pulmonary hypertension. Though sometimes not directly related to either the cardiac defect or surgery, specific problems involving different organs may alter the normal postoperative period. Neurologic, pneumologic, renal, gastrointestinal and infective complications are discussed separately.

[The ghost intraventricular obstruction. Its detection in the right ventricle by Doppler color]. / Obstrucción intraventricular fantasma. Su detección con Doppler color en ventrículo derecho.

INTRODUCTION: The Color Doppler echocardiography is a method of choice to detect the regional acceleration of flow in the left ventricle. The color study confirm it also in the right ventricle of infants with diseases other than hypertrophic cardiomyopathy. MATERIAL: We centered our attention in this finding observed in 16 infants with a mean age of 2.5 months (3 of whom were neonates) with several congenital heart malformations: 9 immediately after surgical ventricular septal defect closure (A-V canal type in 6, and isolated in 3); 2 with pulmonary atresia+intact septum+reconstruction of right ventricular-pulmonary artery connection; 2 with severe aortic stenosis; 1 with a double outlet right ventricle+pulmonary band; 1 with Jatene operation; finally 1 after balloon pulmonary valvuloplasty. RESULTS: The phenomenon consisted in a late Doppler systolic signal in the right ventricular apex whose velocity ranged between 1.5 and 2.9 m/s. The jet was directed towards the tricuspid orifice, with a normal pulmonary flow. The turbulence was easily viewed in the apical four chambers and subcostal sagittal plane of the right ventricle, the spectral signal showed a morphology with left side concavity. An erroneous diagnosis of apical ventricular septal defect was suggested in 2 cases in whom the left ventricular angiogram performed before treatment excluded the lesion. CONCLUSION: The dynamic nature of the jet suggest that an asynchronous order of myocardial contraction in hypertrophic right ventricule play a role in its genesis. With the increasing use of Doppler color technique, this finding can be observed with greater frequency in neonates and infants with operated or unoperated heart diseases and right ventricular hypertrophy. Apical ventricular septal defect will be included in the differential diagnosis.

[Redundancy of the interatrial septum without associated congenital cardiopathy. Its prenatal echocardiographic diagnosis and follow-up]. / Redundancia del septo interatrial sin cardiopatía congénita asociada. Diagnóstico ecocardiográfico prenatal y seguimiento.

INTRODUCTION AND OBJECTIVE: Aneurysm of the atrial septum at the level of the foramen ovale is reported commonly at the routine fetal echocardiographic examination. Cases with entire involvement of the atrial septum have been exceptionally reported. We described the data concerning the prenatal detection of a total redundancy of the atrial septum in 6 cases without congenital heart disease. METHODS: The gestational age was between 28 and 38 weeks, and none of the fetuses had evidence of heart defect. A routine fetal echocardiography was made in all the cases. Rhythm disturbances were studied by M mode. During the first three months of life, a cardiologic control was made in all the cases. RESULTS: The large displacement of the atrial septum towards the left atrium was clearly visualized in four-chambers view; by using Pulsed Doppler and Color flow mapping, the pulmonary venous return and mitral orifice flow were not impaired. Premature atrial beats were found in two fetuses and no hemodynamic disfunction was observed in all entire study group. Postnatal echocardiographic control was normal in all patients. The atrial septum becomes completely rectified with normal patency of the foramen ovale membrane in the newborn studies. The supraventricular extrasystoles were confirmed in the neonatal period. In both, the arrhythmia disappeared in a few days after delivery without treatment. CONCLUSIONS: Despite the benign follow-up in our cases, the unexpected presence of an extremely redundant atrial septum, leads to a close prenatal attention particularly in the presence of rhythm disturbance. The pathologic substrate of this anomaly might be an intrinsic alteration of the myocardial structure of the septum like seems demonstrated in the aneurysm circumscribed to area of the fossa ovalis [correction of fosa ovale]. The increase in the left atrial pressure after birth will produce a normal 2-D echo patterns in the newborn period.

[Balloon pulmonary valvuloplasty in the neonatal period. The clinical and echocardiographic effects]. / Valvuloplastia pulmonar con balón en el período neonatal. Efectos clínicos y ecocardiográficos.

AIM: To analyze the efficacy of balloon pulmonary valvuloplasty (BPV) as the elective treatment for neonatal critical pulmonary valvar stenosis (PVS). MATERIALS AND METHODS: The results of clinical and echocardiographic features before and after the BPV were reviewed in 29 neonates (18+/-12 days of life). Different hemodynamic and 2-D color Doppler echocardiographic were evaluated. The BPV result was classified as favourable if no other balloon or surgical therapy was required to normalise pulmonary flow and achieve a sustained right ventricle-pulmonary artery (RV-PA) Doppler gradient below 40 mmHg. It was considered unfavourable if the neonate died, needed surgery or redilation and/or the RV-PA Doppler gradient was > or =40 mm Hg. The study developed in three phases: pre BPV immediate post BPV until the hospital discharge (14+/-11 days), and in the mid-term follow-up of between 8 and 96 months (51+/-31 months). RESULTS: Mortality was not registered with BPV. The RV/left ventricular systolic pressure decreased from 1.4+/-0.3 to 0.8+/-0.3 (p<0.01) as a consequence of the dilation, and the the systemic oxygen saturation increased from 85 +/-12 to 92+/-6% (p<0.01). The RV-PA Doppler gradient diminished from 86+/-18 to 28+/-16 mm Hg immediately after BPV (p<0.01) and was registered at 13+/-6 mm Hg in the follow-up (n = 24). The RV-PA junction Z value grew from -1.25+/-0.9 before valvuloplasty to -0.51 +/-0.7 at the final echocardiogram (p<0.01). No changes in the tricuspid diameter were detected between both periods of time. Five neonates obtained unsatisfactory results: 4 in the immediate post BPV (systemic-pulmonary artery shunt 2, transannular patch 2), and 1 in the mid-term follow-up (valvectomy + transannular patch). The actuarial curve reflects that 82,7% of the patients were free form reinterventions at 8 years. CONCLUSIONS: BPV is safe and effective to relief PVS in the neonate. The balloon promotes advantageous changes in both, pulmonary annulus and the right ventricle. In addition, the RV-PA Doppler gradient observations in the follow-up, support the expectation that the BPV is a "curative" therapy.

[Clinical and echocardiographic impact of neonatal aortic valvuloplasty]. / Impacto clínico y ecocardiográfico de la valvuloplastia aórtica neonatal.

OBJECTIVES: The selection of patients for balloon aortic valvuloplasty is a vital clinical challenge in neonatal aortic stenosis. The present study was designed to establish possible predicting factors for poor results after balloon therapy. PATIENTS AND METHODS: 2D echocardiographic, Doppler color and clinical variables (grade of cardiac failure, aortic "anulus", mitral anulus, left ventricular diameter, ascending aorta, instantaneous Doppler gradient, and grade of aortic regurgitation) were analyzed in 32 neonates submitted to balloon dilation at 22 +/- 18 days of life. The evaluations were conducted during three periods in respect to balloon therapy (A: before; B: immediately after, and C: mid-term follow-up). The outcome in both, B and C periods was defined as favorable (all survivors with Doppler gradient < or = 70 mmHg and no other intervention on the valve) and unfavorable (death, first stage of univentricularization, valvular replacement or survivors with Doppler gradient > 70 mmHg). Mean values between subgroups were compared by Wilcoxon test; unconditional logistic regression was used to analyze the impact of cardiac failure and anatomic variables (continuous, categorized or Z) on the outcome. RESULTS: The Doppler gradient decreased from 70 +/- 28 to 34 +/- 14 mmHg after the valvuloplasty, and no changes were detected in the follow-up period (36 +/- 8 mmHg). An immediate favorable result was obtained in 72% of the patients; its consisted of 50% in period C. Nine neonates had an immediate unfavorable outcome (6 deaths and 3 with Norwood operation). In the follow-up, three patients had valvular replacement, one patient Doppler gradient > 70 mmHg and one patient with left ventricular endomyocardial abnormalities died. The severe cardiac failure (odds ratio: 33; CL 2.4-443; p = 0.008) and all categorized anatomic variables (aortic "anulus" < or = 6 mm; mitral anulus < or = 9 mm; ascending aorta < or = 8 mm; left ventricular diameter < or = 13 mm) were related with the immediate poor outcome. At 7.6 years, survival and freedom with no valvular replacement nor reintervention probability rates were 83% and 67%, respectively. CONCLUSIONS: 2D echo Doppler provides essential information about the anatomic and functional lesions coexisting with severe or critical aortic stenosis in neonates. Patients with left heart hypoplasia and severe heart failure should not be candidates for balloon valvuloplasty. The degree of residual aortic regurgitation and endomyocardial abnormalities of the left ventricle play an important role in the mid-term follow-up.

[The bidirectional Glenn operation in 100 cases with complex congenital heat diseases: factors influencing surgical results]. / La operación de Glenn bidirectional en 100 casos con cardiopatías congénitas complejas: factores determinantes del resultado quirúrgico.

OBJECTIVE: The bidirectional Glenn shunt is a well established surgical technique in children with complex congenital heart disease. The present study is a retrospective analysis of patients undergoing this operation in order to assess the influence of different variables in the outcome. PATIENTS AND METHOD: From December 1990 to June 2000, 100 patients received a bidirectional Glenn shunt. Two groups were defined, based on the outcome. Group A (n = 15, unsuccessfully result) including death and need to reoperate, and Group B (n = 85, patients with good outcome). RESULTS: The mortality was 8%. Eight patients were reoperated at follow-up. The survivors were followed for a mean of 3.5 years. Mean pulmonary artery pressure 7 mmHg was a factor associated with poor clinical progress. Other variables (age less than 1 year, excessive pulmonary blood flow, double Glenn operation, significant anatomic anomalies, and arrythmias), were also associated with outcome. Significant variations were observed in the time of by-pass or the need for aortic clamp in cases with simultaneous operative repair of pulmonary branch stenosis. Actuarial survival rate, most more than 1 year was 92%, and freedom from reoperation at 3 years was 90%. CONCLUSIONS: The bidirectional Glenn shunt is an excellent palliation in patients with functionally single ventricle. Mean pulmonary artery pressure was the most important variable related with the outcome. We are encouraged to continue with tendency to perform bidirectional Glenn shunt preferably early, avoiding, whenever possible, previous palliative surgery.

[Non-surgical treatment of aortic coarctation and recoarctation]. / Tratamiento no quirúrgico de la coartación y recoartación de aorta.

OBJECTIVES: To analyze the results of nonsurgical treatment of aortic coarctation and recoarctation by evaluating the results of each technique, and its complications and outcome. PATIENTS AND METHODS: The results were as follows: 51 children underwent balloon dilatation due to recoarctation (86.5 %); two underwent dilation of a native coarctation (3.3 %) and six underwent stent implantation for recoarctation (10 %). Age ranged from 2 to 236 months (109 +/- 63.45 months) with a follow-up of between 1 and 156 months (38.87 +/-32.96 months). RESULTS: The mean predilatation gradient in children with recoarctation was 34 +/- 11.62 mmHg, which decreased to 11 +/- 5.38 mm Hg (p < 0.0001). In 12 patients (20.3 %) effective dilatation was not achieved. The size of the stenosis was 6.7 +/- 2.35 mm predilatation, which increased to 9.3 +/- 3.10 mm (p < 0.0001) after dilatation with a percentage increase of 50.97. There were very few complications. Six children required subsequent redilatation. The experience with stent showed a mean gradient of 32.83 +/- 10.62 mm Hg, which decreased to 7.3 +/- 3.8 mm Hg (p < 0.0001) with a balloon/stenosis ratio of 1.94. CONCLUSIONS: We conclude that the interventionist technique is highly effective in both native coarctation and recoarctation in the short term, as well as subsequently, with very few complications. The stent technique produces equally good results in older children, which is promising for the future.

[Clinical consensus on respiratory syncytial virus (RSV) infection prophylaxis and the use of palivizumab in paediatric cardiology.]. / Consenso clínico sobre la profilaxis de la infección por virus respiratorio sincitial y el uso del palivizumab en cardiología pediátrica.

BACKGROUND: Following the results of the CIVIC study, the SECPCC proposes to revise its recommendations for the prevention of RSV, taking into account the new evidence, as well as the preventive experience of paediatric cardiologists. For this purpose a structured method of professional consensus has been chosen. OBJECTIVES: To develop a Spanish clinical consensus on preventing infection by RSV under the auspices of the Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas. METHODS: Delphi Consensus modified in two rounds. The study was conducted in four phases: 1) constitution of a Scientific Committee for bibliographic review and submission of the recommendations for discussion, 2) constitution of an Expert Panel with 75 representatives in the speciality, 3) postal survey organised in two rounds and intermediate processing of opinions, and issuing of a report to the panellists, and 4) discussion of the results in a face-to-face meeting of the Scientific Committee. RESULTS: Consensus was reached on 54 of the 70 preventive recommendations analysed. With respect to the 16 remaining issues, no consensus was reached, due to differences in professional opinion and the absence of established criteria among the majority of the experts. CONCLUSIONS: A set of recommendations for RSV prophylaxis in cardiology was developed and updated, rated in accordance with the degree of professional consensus on which they were based. These can be considered valid until such time as new scientific information emerges that warrants a further review.