Pulse oximetry screening for critical congenital heart defects.

BACKGROUND: Health outcomes are improved when newborn babies with critical congenital heart defects (CCHDs) are detected before acute cardiovascular collapse. The main screening tests used to identify these babies include prenatal ultrasonography and postnatal clinical examination; however, even though both of these methods are available, a significant proportion of babies are still missed. Routine pulse oximetry has been reported as an additional screening test that can potentially improve detection of CCHD. OBJECTIVES: • To determine the diagnostic accuracy of pulse oximetry as a screening method for detection of CCHD in asymptomatic newborn infants• To assess potential sources of heterogeneity, including:○ characteristics of the population: inclusion or exclusion of antenatally detected congenital heart defects;○ timing of testing: < 24 hours versus ≥ 24 hours after birth;○ site of testing: right hand and foot (pre-ductal and post-ductal) versus foot only (post-ductal);○ oxygen saturation: functional versus fractional;○ study design: retrospective versus prospective design, consecutive versus non-consecutive series; and○ risk of bias for the "flow and timing" domain of QUADAS-2. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL; 2017, Issue 2) in the Cochrane Library and the following databases: MEDLINE, Embase, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), and Health Services Research Projects in Progress (HSRProj), up to March 2017. We searched the reference lists of all included articles and relevant systematic reviews to identify additional studies not found through the electronic search. We applied no language restrictions. SELECTION CRITERIA: We selected studies that met predefined criteria for design, population, tests, and outcomes. We included cross-sectional and cohort studies assessing the diagnostic accuracy of pulse oximetry screening for diagnosis of CCHD in term and late preterm asymptomatic newborn infants. We considered all protocols of pulse oximetry screening (eg, different saturation thresholds to define abnormality, post-ductal only or pre-ductal and post-ductal measurements, test timing less than or greater than 24 hours). Reference standards were diagnostic echocardiography (echocardiogram) and clinical follow-up, including postmortem findings, mortality, and congenital anomaly databases. DATA COLLECTION AND ANALYSIS: We extracted accuracy data for the threshold used in primary studies. We explored between-study variability and correlation between indices visually through use of forest and receiver operating characteristic (ROC) plots. We assessed risk of bias in included studies using the QUADAS-2 tool. We used the bivariate model to calculate random-effects pooled sensitivity and specificity values. We investigated sources of heterogeneity using subgroup analyses and meta-regression. MAIN RESULTS: Twenty-one studies met our inclusion criteria (N = 457,202 participants). Nineteen studies provided data for the primary analysis (oxygen saturation threshold < 95% or ≤ 95%; N = 436,758 participants). The overall sensitivity of pulse oximetry for detection of CCHD was 76.3% (95% confidence interval [CI] 69.5 to 82.0) (low certainty of the evidence). Specificity was 99.9% (95% CI 99.7 to 99.9), with a false-positive rate of 0.14% (95% CI 0.07 to 0.22) (high certainty of the evidence). Summary positive and negative likelihood ratios were 535.6 (95% CI 280.3 to 1023.4) and 0.24 (95% CI 0.18 to 0.31), respectively. These results showed that out of 10,000 apparently healthy late preterm or full-term newborn infants, six will have CCHD (median prevalence in our review). Screening by pulse oximetry will detect five of these infants as having CCHD and will miss one case. In addition, screening by pulse oximetry will falsely identify another 14 infants out of the 10,000 as having suspected CCHD when they do not have it.The false-positive rate for detection of CCHD was lower when newborn pulse oximetry was performed longer than 24 hours after birth than when it was performed within 24 hours (0.06%, 95% CI 0.03 to 0.13, vs 0.42%, 95% CI 0.20 to 0.89; P = 0.027).Forest and ROC plots showed greater variability in estimated sensitivity than specificity across studies. We explored heterogeneity by conducting subgroup analyses and meta-regression of inclusion or exclusion of antenatally detected congenital heart defects, timing of testing, and risk of bias for the "flow and timing" domain of QUADAS-2, and we did not find an explanation for the heterogeneity in sensitivity. AUTHORS' CONCLUSIONS: Pulse oximetry is a highly specific and moderately sensitive test for detection of CCHD with very low false-positive rates. Current evidence supports the introduction of routine screening for CCHD in asymptomatic newborns before discharge from the well-baby nursery.

Cardiorespiratory Function Does Not Improve 2 Years After Posterior Surgical Correction of Adolescent Idiopathic Scoliosis.

STUDY DESIGN: A prospective evaluation of cardiorespiratory function following spinal fusion in adolescent idiopathic scoliosis (AIS). OBJECTIVE: To evaluate the cardiopulmonary function during exercise in patients with severe AIS, before and 2 years after undergoing a posterior spinal fusion. SUMMARY OF BACKGROUND DATA: After surgical correction of scoliosis, a greater cardiorespiratory adaptation to exercise would be expected from correction of the rib cage associated with the spine deformity. However, there is no clear evidence regarding whether tolerance to maximum exercise improves in the medium term after surgery in patients with severe curves. METHODS: We include patients with AIS proposed for posterior surgical correction aging between 12 and 17 years. Every patient had a Cobb angle >45° and a Lenke type 1A scoliosis. Cardiac and respiratory functional measures, such as heart rate and blood pressure, maximum oxygen consumption (VO2max), eliminated volume of carbon dioxide (VCO2), quotient between ventilation and volume of exhaled carbon dioxide (VE/CO2), respiratory exchange rate, ventilatory capacity at maximal exercise (VEmax), were recorded before and 2 years after surgery. RESULTS: Twenty patients were included in our study, 15 girls and 5 boys, with an average age of 13 years. The main scoliotic curve was corrected in the coronal plane in an average of 71.9%. The maximal aerobic power expressed by body weight normalized VO2max was found preoperatively to have an average of 30.9 ±â€Š6.2 mL/kg/minute, indicating a poor aerobic capacity, which did not improve at final follow-up, decreasing to a mean value of 29.3 ±â€Š5.7 but without statistical significance. However, the percentages of curve correction showed a statistically significant correlation with VO2max (r = 0.534; P < 0.05). CONCLUSION: Patients with severe adolescent idiopathic scoliosis Lenke type 1A showed limited cardiorespiratory tolerance to maximum exercise that did not improve 2 years after surgery. LEVEL OF EVIDENCE: 3.

[Absent right superior vena cava with left superior vena cava draining to an unroofed coronary sinus]. / Ausencia de vena cava superior derecha y vena cava superior izquierda con drenaje en el seno coronario sin techo.

We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.

[Single-stage Fontan procedure: early and late outcome in 124 patients]. / Operación modificada de Fontan o variantes efectuadas en un solo tiempo quirúrgico. Determinantes de la mortalidad.

INTRODUCTION AND OBJECTIVES: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period. MATERIAL AND METHODS: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified. RESULTS: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years. CONCLUSIONS: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.

[Percutaneous catheter closure of atrial septal defect. Short-term and mid-term results]. / Cierre percutáneo de la comunicación interauricular. Resultados a corto y mediano plazo.

INTRODUCTION: Percutaneous closure of atrial septal defects (ASD) is becoming more frequent. PATIENTS AND METHOD: From October 1997 to October 2002, 209 patients, age 0.4-70 (mean 19.5) years, were catheterized to close an ASD or patent foramen ovale (PFO). Transesophageal echocardiography was performed simultaneously in all patients. Two hundred and six patients had ASD (25 multiple ASDs) and 3 had PFO. Three devices were used, the Amplatzer Atrial Septal Occluder (ASO), CardioSeal (CS), and Starflex (SF). RESULTS: Device implantation was achieved in 181 patients (87%) but had to be abandoned in 28 patients, generally because the ASD was too large. One hundred and seventy-four ASOs were implanted in 172 patients with ASD (2 ASOs were implanted in 2 patients with double ASD) and CS/SF in 9 patients (3 patients with PFO and 6 with ASD). The procedure was effective in 166/172 (96%) ASO implantations and in 8/9 (89%) CS/SF implantations. The procedure was unsuccessful in 7 patients and the device had to be removed (6 ASO and 1 SF). The occlusion rate with ASO was 88% after 24 hours, 91% after 1 month, 95% after 1 year, 97% after 2 years, and 100% after 4 and 5 years. All defects treated with SF/CS were closed successfully after 24 hours. In one case the ASO device was embolized to the aorta. In the first month after ASO implantation, supraventricular tachycardia appeared in 2 patients and transient left ventricular failure in 2 patients. No late complications were observed. CONCLUSION: Percutaneous catheter closure of selected types of ASD using the Amplatzer Atrial Septal Occluder, CardioSeal, or Starflex should be offered to patients as non-surgical alternative. The type of device used depends on the defect size and morphology as well as the surgeon's experience. The presence of multiple defects does not exclude the possibility of a successful percutaneous catheter closure.

Experiencia con la prótesis de pericardio bovino Carpentier-Edwards® en posición pulmonar en pacientes con cardiopatías congénitas / Experience with Carpentier Edwards® bovine pericardium implantations in pulmonary position in patients with congenital heart diseases

Introducción. El número de pacientes con cardiopatías complejas corregidas en la infancia que necesitan una sustitución valvular pulmonar para restaurar la competencia o solucionar la estenosis del tracto de salida de ventrículo derecho ha aumentado en los últimos años. El injerto ideal continúa siendo motivo de controversia. En el servicio de cardiocirugía del Hospital Ramón y Cajal de España, se comenzó a utilizar prótesis de pericardio bovina de Carpentier-Edwards siendo el objetivo de este estudio su evaluación a corto y medio plazo. Materiales y Métodos. Entre enero de 2004 y mayo de 2010 fueron intervenidos 42 pacientes para sustitución valvular pulmonar mediante prótesis de pericardio bovino. El estudio fue ambispectivo con prospección durante los dos últimos años. Resultados. La mediana de la edad fue de 20,96 años (amplitud intercuartil 10,5 años). El número medio de cirugías previas fue de 1,9±0,9 siendo el tiempo medio entre la última cirugía y la implantación de la prótesis de 17,2±7 años. Las indicaciones quirúrgicas fueron: disfunción del ventrículo derecho (45%), su dilatación progresiva (38%), arritmias ventriculares (14%) y síncopes (3%). La mortalidad precoz de causa cardiológica fue de dos pacientes. El tiempo medio de seguimiento fue de 2,1±1,4 años (rango entre 0,1 y 6,3 años) estando el 94,3% de ellos en clase funcional I de la New York Heart Association. El gradiente Doppler pico transprotésico por ecocardiografía fue de 18,5±17 mm Hg. No se observaron cambios degenerativos ni ningún tipo de deterioro estructural de la prótesis. Conclusiones. La prótesis de pericardio bovino en posición pulmonar presenta excelentes resultados a corto y medio plazo. Sin embargo, es necesario un seguimiento mayor para confirmar lo resultados iniciales respecto a su durabilidad y hemodinamia a largo plazo...

Introduction. In recent years the number of patients with complex congenital heart disease previously corrected in infancy who need a pulmonary valve replacement has increased dramatically. The ideal substitute remains a source of dispute. Nowadays, in the unit of heart surgery of the Hospital Ramon y Cajal in Spain, its being implanting in this position the bovine pericardium Carpentier-Edwards prosthesis. The aim of the study is its short and mediumtermassessment. Material and methods. Between January 2004 and May of 2010, 42 patients have been operated for pulmonary valve replacement with pericardium prosthesis. The study was ambispective, being prospective in its last two years. Results. The median age of the patients was 20.96 years (interquartile 10.5 years). The mean number of surgeries prior to the pulmonary valve replacement was 1.9±0.9, being the mean time between the “corrective” surgery and the prosthetic implantation 17.2±7 years. The main indications for this surgery were: right ventricle dysfunction (45%), progressive dilation of the same ventricle (38%), ventricular arrhythmias (14%) and syncopes (3%). Two patients died in the immediate postoperative due to cardiological causes. The mean follow-up time has been 2.1±1.4 years (0.1-6.3). The 94.3 % of the surviving patients are in functional class I, according to the New York Health Association. The peak Doppler transprosthetic gradient determined by echocardiography was 18.5±17 mm Hg. In the echocardiograpic follow-up there have been neither degenerative changes nor any type of structural deterioration of the prosthesis. Conclusions. The bovine pericardium prosthesis in pulmonary position presents excellent results in the short and medium-term. However, it is necessary a longer follow-up to confirm our initial results regarding its durability and haemodynamics long-term...

The clinical profile of Ebstein's malformation as seen from the fetus to the adult in 52 patients.

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

Túnel aorta-ventrículo izquierdo. Resultados quirúrgicos a largo plazo / Aorti-Left ventricular tunnel long term surgical results

Objetivo: Describir diferentes aspectos relacionados con el diagnóstico y la evolución postoperatoria a largo plazo de cuatro pacientes operados exitosamente de túnel aorta-ventrículo izquierdo (TAVI). Escasas son las publicaciones que detallan la evolución postoperatoria a largo plazo, en especial la que se refiere a la progresión de la regurgitación aórtica residual. Método: Revisamos retrospectivamente las historias clínicas de cuatro pacientes con diagnóstico de TAVI que fueron operados entre 1983 y 1997. Analizamos la información preoperatoria y postoperatoria derivada de los datos clínicos, electrocardiográficos, radiológicos, ecocardiográficos y angio-hemodinámicos. El periodo de seguimiento osciló entre 4.7 y 13 años. Resultados: En un periodo de 14 años, cuatro niños (dos hombres, dos mujeres), fueron sometidos a reparación quirúrgica del TAVI; la media del tiempo de diagnóstico fue de 8.5 años (entre 1 mes y 14 años). Todos los pacientes tenían soplo sitio-diastólico, excepto en un neonato que además, presentó insuficiencia cardiaca congestiva y cianosis en la primera semana de vida. En todos los enfermos se sospechó la presencia de TAVI; sin embargo, sólo en dos se consideró que los datos anatomo-ecocardiográficos 2-D Doppler color fueron inequívocos de la malformación. El estudio angio-hemodinámico confirmó el defecto en tres niños; en el restante, al igual que con el examen ecográfico, se diagnosticó de aneurisma perforado del seno de Valsalva derecho. El túnel fue cerrado en el extremo aórtico con parche de teflón en dos y con sutura directa en los restantes. No hubo mortalidad hospitalaria y la edad media de cirugía fue de 9 años (entre 1.5 meses a 14 años). Durante un periodo de seguimiento de 8.6 años, tres pacientes tienen evidencia clínica y ecocardiográfica de insuficiencia aórtica valvular genuina, sin evidencia de regurgitación a través del TAVI. Todos los pacientes se encuentran asintomáticos al seguimiento. Ningún paciente ha sido reintervenido, y en uno se llevó a cabo recambio valvular aórtico