Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study.

BACKGROUND: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. METHODS: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. RESULTS: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. CONCLUSION: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.

Impact of Interatrial Communication on Left Ventricle Performance in Patients with Significant Post-tricuspid Shunt.

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E'- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.

Recombinant Tissue Plasminogen Activator in the Treatment of Neonates with Intracardiac and Great Vessels Thrombosis.

Life-threatening intracardiac and great vessels thrombi are rare in neonates. Recombinant tissue plasminogen activator (rTPA) is used in adults to stimulate fibrinolysis and facilitate thrombus resolution. Its use in neonates, along with heparin, remains controversial because of potential risk of serious bleeding. We aim to present our experience with the use of thrombolytic agents in seven neonates and young infants. In a retrospective study, over a period of 6 years, the medical records of neonates and young infants, who were diagnosed with intracardiac and great vessels thrombi, were reviewed. The following factors were collected: demographic data, primary diagnosis, thrombus site, risk factors, method of diagnosis, thrombolytic and/or anticoagulation agent, route, dose and duration of treatment, complications, and outcome. Six neonates and one 45-day-old infant were analyzed. Age ranged from 5 to 45 days (median age 12 days), and median weight was 2.9 kg (range 0.9-3.8 kg). The thrombi were diagnosed by echocardiography in five and by angiography in two cases. All patients had life-threatening thrombi; four were treated with rTPA (0.5 mg kg(-1) h(-1)) and heparin infusions with complete dissolution of the thrombi, within a median time of 60 h (6-72 h), and without complications. The remaining three patients (two who were premature, at 28 and 34 weeks of gestation, and the third who had a deranged coagulation profile) were treated with unfractionated heparin due to fear of bleeding. The thrombi dissolved in the premature babies (within 2 weeks and 3 months, respectively) but embolized and resulted in the death of the third infant after 2 weeks of treatment. The current case series confirmed the effectiveness and safety of intravenous rTPA infusion, at the dosages used, in neonates and young infants with life-threatening thrombi.

Recanalization of arterial duct is feasible, effective and its potential risks are treatable.

BACKGROUND: Recanalization of arterial duct (AD) is rarely needed. OBJECTIVE: The aim of this study is to report our experience regarding the feasibility and effectiveness of arterial duct recanalization in three infants and review the relevant literature. METHODS AND RESULTS: We report on three patients with decreased pulmonary blood flow after initial palliation. The first patient had pulmonary atresia (PA) and an intact ventricular septum. He needed recanalization of the AD after pulmonary valve perforation and dilatation. The second patient had PA and ventricular septal defect (VSD). His AD originated from the left subclavian artery. He required AD recanalization after palliation with a central shunt and clipping of the duct. During intervention he developed a thrombus in the stent, which was treated successfully using thrombolytic treatment. The third patient had PA and VSD. The arterial duct originated from the left subclavian artery and was recanalized after spontaneous closure despite prostaglandin infusion. During the procedure the patient had severe desaturation and bradycardia requiring resuscitation fortwo minutes. All infants had successful arterial duct recanalization and stenting. Additionally, they were clinically stable during follow-up. CONCLUSION: Arterial duct recanalization and stenting is a feasible and effective procedure in selected cases, and its risks are treatable. Long-term studies are required.

Combined semilunar valve stenoses in neonates: management approaches and literature review.

Combination of right and left ventricular outflow tracts obstruction is extremely rare. Neonates with combined aortic stenosis (AS) and pulmonary stenosis (PS) present in critical condition and required urgent treatment. The management approach is not well defined. We report five female neonates with combined AS and PS presented to our institute in the last 5 years, age (1-18 days), weight (2.2-3.4 kg). Two had associated muscular ventricular septal defects. The mean Doppler gradient across the aortic valve (AV) was 73 mmHg (53-105 mmHg) and across the pulmonary valve was 62 mmHg (44-76 mmHg). Three had balloon dilatation and one surgical repair. The fifth patient was managed conservatively, but had sudden cardiac death at age of 3 months. One patient arrived in shock and sepsis, underwent emergency balloon dilation of the AV in the ICU. Despite decreasing the gradient, she died next day after the procedure. The surviving children were well at median follow-up age of 3.4 years. This is an extremely rare combination which needs early intervention. The management approach is not well defined. Interventional catheterization is possibly the better option.

Cardiac rhabdomyoma mimicking haemodynamics of hypoplastic left heart syndrome.

Cardiac rhabdomyomas are rare and often regress spontaneously. However, the management of rhabdomyoma with severe inflow and outflow obstructions is a challenge. An infant with a massive left ventricular rhabdomyoma mimicking the haemodynamics of hypoplastic left heart syndrome is reported. Surgery could not be contemplated because the mitral valve leaflets and chordae were imbedded in the tumour mass. The arterial duct (AD) was kept open to perfuse the systemic circulation and palliations with pulmonary artery branch banding and AD stenting were planned. However; while waiting for spontaneous regression of the tumour, the child died of circulatory collapse when 4 weeks old.

Screening of congenital heart disease patients using multiplex ligation-dependent probe amplification: early diagnosis of syndromic patients.

Recurrent copy number variants (CNVs) are found in a significant proportion of patients with congenital heart disease (CHD) and some of these CNVs are associated with other developmental defects. In some syndromic patients, CHD may be the first presenting symptom, thus screening of patients with CHD for CNVs in specific genomic regions may lead to early diagnosis and awareness of extracardiac symptoms. We designed a multiplex ligation-dependent probe amplification (MLPA) assay specifically for screening of CHD patients. The MLPA assay allows for simultaneous analysis of CNVs in 25 genomic regions previously associated with CHD. We screened blood samples from 402 CHD patients and identified 14 rare CNVs in 13 (3.2%) patients. Five CNVs were de novo and six where inherited from a healthy parent. The MLPA screen led to early syndrome diagnosis in two of these patients. We conclude that the MLPA assay detects clinically relevant CNVs and suggest that it could be used within pediatric cardiology as a first tier screen to detect clinically relevant CNVs and identify syndromic patients at an early stage.

Quantification of left-to-right shunt through patent ductus arteriosus by colour Doppler in children admitted for a device closure.

PURPOSE: Our animal model suggests that quantification of ductal flow from colour Doppler pixels is possible. We aimed to clarify whether this method can be used to determine a clinically significant ductal shunt in children. METHODS: We retrospectively quantified ductal flow from saved images from 20 children who had been admitted for device occlusion of patent ductus arteriosus. Colour Doppler images over the main stem of the pulmonary artery were obtained in longitudinal cross-sections. The colour pixel percentages during diastole, representing ductal flow, were correlated with the documented shunt, measured invasively according to Fick's principle. RESULTS: The ratio of pulmonary to systemic flow correlated best with the sum of the percentages of green colour pixels (r = 0.73, r2 = 0.54, p < 0.001). When the shunt was 1.5:1 or more, 12 out of 13 infants had 50% or more of the region of interest covered with green pixels--sensitivity 92%, specificity 71%. The correlation between ductal diameter and pulmonary-to-systemic flow ratio was less significant (r = 0.6, r2 = 0.37, p < 0.03). CONCLUSIONS: We conclude that clinically significant shunts with pulmonary-to-systemic flow ratio over 1.5 can be diagnosed with this method where neither the size of the patient nor echocardiographic settings seem to be critical. The method could be used to provide an objective indication for ductal closure, but further prospective studies in children are needed to verify the power of the method.

Cardiac Tamponade-Associated Dense Deposit Disease: A Case Report and Review of the Literature.

Pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. It can be associated with various cardiac and non-cardiac disorders. Dense deposit disease (DDD) is a rare kidney disease caused by uncontrolled activation of the alternative complement pathway. We are reporting a seven-year-old male child who was diagnosed to have DDD approved by renal biopsy and presented with shortness of breath, cough, and fever. Chest X-ray displayed cardiomegaly. Thereafter, echocardiography showed massive pericardial effusion and left ventricle compression with a risk for cardiac tamponade. He subsequently underwent pericardiocentesis with the removal of 450 ml of pericardial fluid. The patient's edema was not correlated with the described amount of drained pericardial fluid. To the best of our knowledge, this is the first reported case of significant pericardial effusion carrying the risk of cardiac tamponade associated with DDD. With this report, we would like to highlight the importance of cardiac assessment in patients with DDD, in particular those with nephrotic range proteinuria who present with cardiac symptoms and cardiomegaly.

Late coronary complications after arterial switch operation and their treatment.

OBJECTIVES: To report the late coronary complications and their treatment after arterial switch operation (ASO). BACKGROUND: Asymptomatic patients after ASO may have coronary ostial stenosis or obstruction. METHODS: Since 1980, 279 patients were operated with ASO. At the time of preparing this article, selective follow-up coronary angiograms were done on 81 patients. RESULTS: Coronary stenosis was found in six patients. A 6-year-old patient with left coronary artery (LCA) ostial stenosis and a 9-year-old patient with conus branch occlusion had good collaterals without a need for further treatment. One patient with LCA obstruction, myocardial infarction, and left ventricular failure was operated with osteoplasty at age of 16 years. In three essentially asymptomatic patients, stenting of LCA ostium stenosis was done: in two of them with drug-eluting stents at 9 and 10 years of age and in one with bare-metal stent at 18 years of age. One of these patients was earlier treated with balloon dilatation at 5 years of age which caused intimal dissection. CONCLUSIONS: Asymptomatic patients with an uneventful course after ASO may have coronary obstruction. This necessitates follow-up coronary evaluation in all patients. Stenting of the coronary arteries is an option for treatment.

Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study.

Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live-borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990-2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox-logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live-borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live-borns who underwent surgery increased between time periods.

Atrial septal defect: a diagnostic approach.

A simple objective screening method for diagnosis of the atrial septal defect (ASD) is needed. Acoustic signals were collected from 61 children with ASD and 60 with a physiological murmur. The second heart sound (S2) and the spectrum of systolic murmur were analysed. A statistical model was designed using stepwise logistic regression analysis. Significant variables distinguishing pathological form normal findings were the interval between the first heart sound and the beginning of systolic murmur or the respiratory variation of S2, and the frequency of the murmur at its maximum intensity. The area under the ROC curve was 0.922; indicating very good fit of the model and the confidence interval was 0.872-0.971. The sensitivity of the model was 91% and the specificity 73%. The analysis of acoustic findings from the heart is a valuable tool in diagnosing ASD. The next step will be automating this process.

Percutaneous closure of atrial septal defect with situs solitus and dextrocardia.

Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia.

Paediatricians' referral preference of patients with embolised intravascular foreign bodies: a survey-based study.

BACKGROUND: Central line insertion is a routine procedure in medical practice. Dislodgement of lines into the vascular system is a rare complication. We noticed that paediatric health care providers (PHCP) contact the cardiac or general paediatric surgeon for extraction of dislodged lines more frequently than using the less invasive percutaneous approach. AIM: To study the referral preference of PHCP for patient with embolised intravascular foreign bodies. METHODS: A questionnaire with three questions was distributed to PHCP of all paediatric subspecialties, including surgery, in two tertiary care centres. The questions were about the total number of patients seen with central line, experience with complications, and preferred specialty for removal of dislodged central lines. RESULTS: The questionnaire was distributed to 128 professionals. The response rate was 79% (n=101). Incomplete answers (n=14) were excluded. The grades of responders were senior consultants 18%, junior consultants 38%, and residents 43%. Thirty nine percent of care providers experienced dislodgement or fragmentation of central lines. The majority (82%) prefer to refer the patients for surgical removal. CONCLUSIONS: Most PHCP in the selected hospitals prefer to refer patients with embolised foreign bodies in the vascular system for surgical removal. The local health policy should be updated for the use of the alternative percutaneous approach.

High prevalence rate of left superior vena cava determined by echocardiography in patients with congenital heart disease in Saudi Arabia.

BACKGROUND: Persistent left superior vena cava (LSVC) is one of the common anomalies of the systemic veins. Its prevalence is 0.1-0.3% in the general population and is more common with congenital heart disease (CHD). The importance of detecting persistent LSVC prior to cardiac surgery is paramount for systemic veins cannulations. AIM: The aim was to evaluate the prevalence of persistent LSVC in patients with CHD in Saudi Arabia. METHODS: All patients referred to our institution had echocardiography. All complete studies were reviewed for the presence of persistent LSVC. A computerized database was created including the demographic data, CHD diagnoses, and the presence of persistent LSVC. RESULTS: A total of 2,042 were examined with an age range of 1 day to 16 years. The complete echocardiographic studies were 1,832 (90%) of whom 738 (40%) patients had CHD. The prevalence of persistent LSVC in patients with CHD was 7.8% (OR 9.26, 95% CI 4.7-18.2, p<0.001). The most common cardiac defect associated with persistent LSVC was complete atrioventricular septal defect (AVSD); all patients with AVSD had Down syndrome. The total number of patients with AVSD was 41, and persistent LSVC was found in 11 (26%) of them (odds ratio 5.1, 95% CI 2.4-10.8, p<0.001). CONCLUSIONS: The prevalence of persistent LSVC in the current population is almost double the reported prevalence obtained using the same echocardiographic screening tool.

Infections as a stimulus for coronary occlusion, obstruction, or acute coronary syndromes.

BACKGROUND: Atherosclerosis is considered to be an inflammatory disease. Infections are a significant cause of inflammation. Acute infections might precipitate acute coronary syndromes (ACS) whereas chronic infections might be stimuli for the development of atherosclerosis. METHODS: Coronary angiograms were done on 211 of 335 patients with ACS and the percentage of coronary obstruction was determined. Serum antibody levels to Chlamydia pneumoniae, C. pneumoniae heat shock protein 60 (CpnHSP60), human heat shock protein 60 (hHSP60), enterovirus (EV), herpes simplex virus (HSV), cytomegalovirus (CMV), and two major periodontal pathogens, Aggregatibacter actinomycetemcomitans and Porphyromonas gingivalis, were measured in healthy controls (n = 355) and all patients. RESULTS: Serum antibody levels to periodontal pathogens did not correlate with ACS. However, IgA-class antibody levels to Aggregatibacter actinomycetemcomitans (p = 0.021), CpnHSP60 (p = 0.048) an hHSP60 (p = 0.038) were higher in patients with coronary occlusion or obstruction compared to those without any obstruction. Odds ratios for coronary changes in the highest quartile as compared to the lower quartiles were for A. actinomycetemcomitans IgA 7.84 (95% CI 1.02-60.39, p = 0.048), for CpnHSP60 IgA 8.61 (1.12-65.89, p = 0.038), and for human HSP60 IgA 3.51 (0.79-15.69, p = 0.100). CONCLUSIONS: We have previously reported that EV and HSV titres correlated significantly to acute coronary events. They do not correlate to the degree of coronary obstruction as shown here. However, infection by A. actinomycetemcomitans or C. pneumoniae or host response against them associated with coronary obstruction. Clinical coronary events may arise by the effect of acute infections and obstructing lesions by a chronic inflammatory stimulus.