Search

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.

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.

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.

PurposeOur 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.MethodsWe 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.ResultsThe 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).ConclusionsWe 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.

AIM: To develop an objective diagnostic method that facilitates detection of noncyanotic congenital heart diseases. METHODS: Heart sounds and murmurs were recorded from 60 healthy children and 173 children with noncyanotic congenital heart disease. Time intervals were measured and spectrum of the systolic murmurs analyzed. Stepwise logistic regression analysis was used to distinguish physiological from pathological signals. The receiver operating characteristic (ROC) curve was plotted to show the classification performance of the model and the area under the curve (AUC) was calculated. The probability cut-off points for calculation of sensitivities and specificities were estimated. RESULTS: The distinguishing variables were the interval from the end of the first heart sound (S(1)) and the beginning of the systolic murmur, respiratory variation of the splitting of the second heart sound, intensity of the systolic murmur, and standard deviation of the interval from the end of the S(1) to the maximum intensity of the murmur. The AUC was 0.95, indicating an excellent classification performance of the model. The sensitivity of 95% and specificity of 72% was achieved at a probability cut-off point of 0.45. Significant cardiac defects were correctly classified. CONCLUSION: Interval measurements and spectral analysis can be used to confirm significant noncyanotic congenital heart diseases. Further development of the method is necessary to detect also insignificant heart defects. (Less)

UNLABELLED Three newborn infants with transposition of the great arteries (TGA) and intact ventricular septum (IVS) developed postnatal persistent pulmonary hypertension of the newborn (PPHN) and were successfully treated with inhaled nitric oxide (iNO). Intervention with balloon atrial septostomy (BAS) was performed in two of the infants before the iNO treatment, but they continued to be severely hypoxic with metabolic acidosis. However, the iNO immediately improved oxygenation and the clinical condition. The third neonate had a moderately large atrial communication and echocardiographic signs of PPHN. He received iNO before BAS with dramatic clinical improvement, which therefore postponed BAS. CONCLUSION Early diagnosis of PPHN and treatment with iNO may improve final outcome in neonates with TGA and IVS. In the presence of moderately large atrial communication and PPHN, treatment with iNO might be considered before BAS.

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.

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 for two 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. (Less)

A full term female infant was born after 41 weeks of gestation, with a birth weight of 3815g and normal Apgar scores (9-10- 10). Immediately after birth tachycardia was noted. An electrocardiogram (ECG) showed a heart rate of 300 beats per minute (bpm) and narrow QRS complexes. The newborn was clinically stable. She was admitted to the neonatal intensive care unit and an intravenous line was inserted for treatment with Adenosine. While preparing for Adenosine infusion her heart rate spontaneously decreased. Her ECG at presentation with normal heart rate is shown in figure1a. On auscultation, a soft systolic murmur was heard. Echocardiography was performed and demonstrated a complete atrio-ventricular septal defect (AVSD) with a large primum defect, large ventricular septal defect, common atrio-ventricular orifice (AV-valve), and balanced size of the right and left ventricles. At around two hours of age the infant started to get frequent, short episodes of supraventricular tachycardia alternating with sinuous rhythm (figure 1b). Prophylactic treatment against recurrent tachy-arrythmia with Digoxin was started (full digitalisation within the first 24 hours and then with a maintenance dose of 5 ?g/kg, B.I.D). The infant was observed for several days and continued to be free of symptoms. She was discharged to her home at five days of age. She was seen five days later and was found to be clinically stable with no tachy-arrhythmia. Her ECG then is shown in figure 2.

To develop a digital algorithm that detects first and second heart sounds, defines the systole and diastole, and characterises the systolic murmur. Heart sounds were recorded in 300 children with a cardiac murmur, using an electronic stethoscope. A Digital algorithm was developed for detection of first and second heart sounds. R-waves and T-waves in the electrocardiography were used as references for detection. The sound signal analysis was carried out using the short-time Fourier transform. The first heart sound detection rate, with reference to the R-wave, was 100% within 0.05-0.2R-R interval. The second heart sound detection rate between the end of the T-wave and the 0.6R-R interval was 97%. The systolic and diastolic phases of the cardiac cycle could be identified. Because of the overlap between heart sounds and murmur a systolic segment between the first and second heart sounds (20-70%) was selected for murmur analysis. The maximum intensity of the systolic murmur, its average frequency, and the mean spectral power were quantified. The frequency at the point with the highest sound intensity in the spectrum and its time from the first heart sound, the highest frequency, and frequency range were also determined. This method will serve as the foundation for computer-based detection of heart sounds and the characterisation of cardiac murmurs.

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.

Background Coarctation of aorta may present as severe heart failure in infants and may lead to myocardial dysfunction. Current evidence supports surgical management of neonatal coarctation. However, it can be precarious in critically sick infants. Aim and objective To investigate the safety, efficacy, immediate and mid-term outcome of percutaneous balloon angioplasty (BAP) for infantile aortic coarctation in critically sick patients. Methods and patients Data of all patients under age of 6 months who underwent balloon angioplasty for coarctation were reviewed. Results Between January 2008 and April 2014, 15 infants were identified with coarctation with a mean weight of 3.4 kg (1.4–5 kg) and median age of 54 days (4–142 days). All patients were critically sick, needed admission in intensive care unit for inotropic or ventilatory support. All underwent successful percutaneous balloon angioplasty with no major complications. The clinical condition and left ventricular function improved leading to discontinuation of prostaglandin and weaning from inotrope and ventilator support. Seven patients underwent elective surgical repair after a mean time of one month, three needed re-dilation and five continued without any further intervention. Conclusions Surgical repair for native neonatal and infantile coarctation is a preferred choice of treatment but it can be challenging in critically sick patients. However, balloon dilation remains a safe and effective temporary palliation for the critically sick patients. Despite of high incidence of restenosis, some patients do not need for mid-term further intervention.

Cardiac rhabdomyomas are rare and often regress spontaneously. However, the management of rhabdomyoma with severe infl ow and outfl ow 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 leafl ets 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.

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.

Background Incidence of congenital heart disease in patients with Down syndrome (DS) is 40%. In the past some have advocated that the cardiac defects in DS should not be repaired, reports had shown outcome comparable to individuals without DS. The aim of the study is to analyze the outcome of cardiac surgery in DS. Methodology We conducted a retrospective study in the last 2 years at our institution. Patients involved were DS less than 13 years who had cardiac surgery. DS with chronic lung disease or unreactive pulmonary hypertension were excluded. We analyzed their demographic data, cardiac lesion, type of surgery, ICU stay, morbidities and mortality. Results 23 patients with DS (11 Females, 13 Males), with median age of 7 months, mean age of 32month. AVSD were ( n = 15) 65%), VSD were ( n = 5) 21%. Postoperatively, 21% were extubated on the same day. 27% had intubation more than 9 days. Almost 30% had a hospital stay of >15 days. Complications were respiratory problems in 45%, arrhythmias in 8% and residual AV valve regurgitation in 8%. Hospital mortality was ( n = 2/23) 8%. Conclusion Whether to operate Down syndrome patients or not remains unclear. Our study showed, significant postoperative complications, lengthy hospital stay and relatively high mortality. Despite of this we feel they should be given the chance of surgery to improve their life quality.

A previously healthy 5-month-old girl presented to ouremergency department with history of fever for 2 weeks,skin rash, red and cracked lips and bilateral redness of theeyes appeared 4 days after fever started. On physicalexamination, the general condition was stable includinggood peripheral perfusion. There was no lymphadenopathy.Chest and abdominal examinations were unremarkable.Electrocardiography, apart from mild sinus tachycardia(HR 110 bpm), was normal with no sign of myocardialischaemia or hypertrophy. Chest X-ray showed cardiomeg-aly. Her blood investigation results were as follows: WBC16.2 9 100/lL (neutrophils 36.7%), normal haemoglobinand platelets 1356 9 100/lL. The kidney function and liverfunction tests were normal. Troponin-T 27 lg/L (lab nor-mal value 14 lg/L) and CKMB 42 lg/L (lab normal value25 lg/L). The diagnosis of Kawasaki disease (KD) wasestablished according to the combination of the clinicalpicture and blood investigations. Echocardiogram wasperformed and showed severe dilatation of left anteriordescending coronary artery with giant fusiform aneurysm(Fig. 1) and intracoronary thrombus (Fig. 2). Additionally,normal myocardial function and moderate pericardialeffusion with no sign of cardiac tamponade. Despite thelate presentation, immunoglobulin infusion was startedcombined with heparin, warfarin and low-dose aspirin. Itwas planned after two days to discontinue heparin. Anelective cardiac computerized tomography (CT) with con-trast injection was performed and shows giant aneurysmaldilatation of right coronary artery (RCA; Fig. 3). A repeatedechocardiography, directed on the RCA, confirms the CTfinding (Fig. 4). The critical evaluation of the initial echo-cardiography examination revealed that the RCA aneurysmwas there from the start but was missed as it is very adjacentto and almost as large as right atrium.Kawasaki disease is also known as mucocutaneouslymph node syndrome. Its diagnosis is usually done throughcombination of certain clinical criteria. One of the mostserious complications of KD is dilatation and fatal aneu-rysms or rupture of coronary arteries in untreated patients.The initial echocardiographic study always evaluates theexistence of coronary artery aneurysms. In the presence of aproximal aneurysm, further coronary artery evaluation isusually performed through CT or magnetic resonancetomography combined with contrast injection and/or car-diac catheterization and angiography.Myocardial ischaemia due to thromboembolism is also awell-reported complication. The guidelines for treatment of

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

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. Keywords: cardiac surgery; clinical practice; transcatheter retrieval (Published: 5 March 2013) Citation: Libyan J Med 2013, 8 : 20495 - http://dx.doi.org/10.3402/ljm.v8i0.20495

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. (C) 2012 Wiley Periodicals, Inc. (Less)

OBJECTIVES:: To report the late coronary complications and their treatment after ASO. BACKGROUND:: Asymptomatic patients after arterial switch operation (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 six year-old patient with left coronary artery (LCA) ostial stenosis and a nine 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 nine and ten 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 five 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. (c) 2010 Wiley-Liss, Inc. (Less)

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.

Congenital coronary artery fistulas are rare anomalies. Most of these fistulas drain to the right side of the heart. They seldom connect to either left atrium or left ventricle. We report a three years old girl in whom an atrial septal defect (ASD) was diagnosed. Echocardiography suggested a possible additional coronary artery fistula to the left ventricle (LV). Before closing the ASD, coronary angiography identified multiple coronary artery fistulas to the left ventricle. Transcatheter closure of the ASD was done successfully. The hemodynamic effect of coronary artery fistula to LV after ASD closure is discussed along with literature review of this extremely rare association.

Three newborn infants with transposition of the great arteries (TGA) and intact ventricular septum (IVS) developed postnatal persistent pulmonary hypertension of the newborn (PPHN) and were successfully treated with inhaled nitric oxide (iNO). Intervention with balloon atrial septostomy (BAS) was performed in two of the infants before the iNO treatment, but they continued to be severely hypoxic with metabolic acidosis. However, the iNO immediately improved oxygenation and the clinical condition. The third neonate had a moderately large atrial communication and echocardiographic signs of PPHN. He received iNO before BAS with dramatic clinical improvement, which therefore postponed BAS.Early diagnosis of PPHN and treatment with iNO may improve final outcome in neonates with TGA and IVS. In the presence of moderately large atrial communication and PPHN, treatment with iNO might be considered before BAS.

Background and objectivesRe-conditioning before cardiac surgery in critically sick children is often needed. We report our experience using tailored management approach in these patients.Methods and patientsThe charts of patients with CHD who judged to have high operative risk were reviewed. Included were patients with: large left to right shunt and ventilation for longer than 2 months, significant left to right shunts at multiple levels combined with malnutrition or recent infection, severely impaired cardiac function needing inotropic support and antifailure medications, recent.RSV infection, severe malnutrition (body weight

Background Very rarely a foreign body in the esophagus may penetrate anteriorly to heart or aorta. Such foreign bodies may cause pericardial effusion and cardiac tamponade. Aim To report that an accidentally swallowed foreign body in the esophagus may penetrate the heart and even cause severe mitral regurgitation. Material and methods A 20 month old girl previously healthy presented to the emergency department with 3 days history of fever, respiratory distress, vomiting and poor feeding. The mother reported that her child had some choking event almost a week before, when the child was admitted to another hospital where diagnosis was made of myocarditis. Anti-failure medications prescribed. On examination she had palpable peripheral pulses, a pansystolic murmur on the cardiac apex, and hepatomegaly. EKG showed sinus tachycardia with left ventricular hypertrophy. Chest X-ray revealed normal cardiac size with lungs congestion and suspicion of possible foreign body. Echocardiography showed dilatation of the left atrium and of the pulmonary veins, severe mitral regurgitation with an echogenic structure in next to the posterior mitral valve leaflet with consensual moving. Chest CT scan revealed a high-density metallic foreign body inside the heart in correspondence of the left atrio-ventricular junction. The foreign body was removed surgically and proved to be a thin shiny metal bar, 2 cm in length. The mitral valve was completely damaged and was replaced by mechanical valve (Carbomedics 18 mm). The postoperative course was uneventful and patient was discharged home with anti-failure medications (frusemide, captopril) and warfarin. She remained asymptomatic one year after surgery. Conclusion The process of diagnosing a foreign body in an infant or a toddler, eroding from the esophagus to the heart was very tricky. The diagnosis was suspected by the history of chocking. The x-ray of the chest showed an abnormal structure on the heart shadow, which was further underlined by the echocardiogram suggesting abnormal structure in the heart. Finally CT angio of heart confirmed a metallic foreign body in the left atrium. Happily, MRI was not done; it may have proved disastrous in such a case. The strategy regarding foreign body management either conservative or by its removal either by cardiac catheterization or surgery, depends on location, size of foreign body as well as on whether the patient is symptomatic and in danger to develop further complications. In our case, as the foreign body was in the left atrium and the patient was very critically ill, we opted to remove it by surgery. Moreover, it was thought that patient might need mitral valve repair but unfortunately the valve was so damaged that we opted for mitral valve replacement.

Video presentation Objectives: Coronary artery bypass graing associated with Ross Procedure performed to 9 years old boy. Dr. Imad Naja; Dr. Nani Najm. Department of Cardiac Sciences, King Abdul-Aziz cardiac Centre, Riyadh, Saudi Arabia. This uncommon presentation of this disease at this age, leads u to share our experience. How to do it is the video presentation of CABG + ROSS performed to a 9 years old boy. Methods: 9 years old boy presented at emergency department with severe chest pain, subsequent Echocardiography showed thickened aortic valve with moderate stenosis. The angiogram showed moderate aortic valve stenosis and severely disease ascending aorta. Result: The surgery was uneventful; it was remarkable the atheromatosis at the level of the ascending aorta, successful outcome, the patient was discharged at the 7th post-operative day. doi:10.1016/j.jsha.2011.02.085 SHA 085. Heart defects in acyanotic pediatric patients referred with heart murmurs Dhafer O. Alqahtani, MD, Ali A. Alakhfash, MD, Omar R Tamimi, MD, Dhafer O. Alqahtani, MD, Ali A. Alakhfash, MD, Omar R Tamimi, MD KACC, Riyadh, Saudi Arabia E-mail address: qahtanidh@ngha.med.sa (D.O. Alqahtani) Objectives: The aim of the study is to explore the prevalence of congenital heart disease in pediatric patients referred to our center solely based on the detection of a heart murmur on routine physical examination. Study design: A retrospective database review was performed for all patients referred to KACC from July 2007 to March 2009 for cardiovascular evaluation because of a heart murmur detected during routine physical examination. This study included all pediatric patients from the neonatal period to 12 years of age who underwent echocardiography in our center. Patients with cyanosis, those with a significant difference in blood pressure between the upper and lower limbs, preterm neonates, patients with acquired heart disease, and syndromic or critically ill patients were excluded from this study. Results: Of the patients in the database, 245 met the inclusion criteria. The median age was 7 months (1 day to 12 years old), and the median weight was 7.85 kg (1.9–54 kg). A normal Abstract for SHA22 195

A 20-month old child presented with severe mitral valve regurgitation and infective endocarditis caused by a swallowed metallic foreign body that penetrated from the oesophagus into the posterior wall of the left atrium. Diagnosis was confirmed by computed tomography (CT) scan (Fig. 1). The foreign body was surgically removed (Fig. 2), and the mitral valve was replaced with a mechanical prosthesis.

Life-threatening intra-cardiovascular thrombi are rare in neonates. Recombinant tissue plasminogen activator (rTPA) which stimulates fibrinolysis, is used in adults to facilitate thrombus resolution. Its use along with heparin in neonates remains controversial because of potential risk of serious bleeding. Our aim was to present our experience with the use of thrombolytic agents in seven neonates and young infants. Methods and patients All neonates and one 45 days old infant, between Jan. 2008 and Jan. 2014, with intracardiac and/or intravascular thrombii who were treated either by thrombolytic agents or by heparin alone were included. The following factors were collected: demographic data, primary diagnosis, and site of thrombus, risk factors, method of diagnosis, route and duration of treatment, dosage of thrombolytic and/or anticoagulation agent, complications and outcome. Results Seven patients were identified. Age range was from five days to 45 days (median age 12 days), median weight 2.9 kg (range 0.9–3.8 kg). The thrombi were diagnosed by echocardiography in five cases and in two by angiography. All patients had life threatening thrombi; four were treated with rTPA and heparin infusions with complete dissolving of the thrombi within short time (2–96 h) without complications. The other three patients (two were premature, 28 and 34 weeks of gestation, and the other had deranged coagulation profile) were treated with unfractionated heparin due to fear of bleeding. The thrombus was dissolved in the premature babies and embolized in the other one, which led to his death. Conclusion Our small case series, confirmed the effectiveness and safety of the used dosage of intravenous infusion of recombinant tissue plasminogen activator in neonates with life threatening thrombi.

Background Percutaneous closure of secondum atrial septal defect (ASD II) in cases of dextrocardia and situs inversus has been reported in only very few cases in the literature. ASD II device closure in case of isolated dextrocardia with situs solitus, according to our knowledge, was never reported before. Objective To describe the unexpected problems encountered in decision making during the deployment of the device in a case of ASD-II with dextrocardia and situs solitus. Methods and results 19 months old girl, a case of situs solitus, dextrocardia, fenestrated ASD-II, moderate pulmonary hypertension since 4 months of age. Echocardiography showed situs solitus, dextrocardia, persistent left superior vena cava draining to coronary sinus and normal pulmonary veins. Fenestrated ASD-II (12 mm size in total) with left to right shunt. The total septal length was 28 mm. Volume loaded right atrium and right ventricle. The procedure During cardiac catheterization we found that in situs solitus with dextrocardia, the left atrium is more superior in relation to the right atrium and the septum seems to be more horizontally oriented. Therefore, when probing the left pulmonary veins, in the lateral view, it appeared much more anteriorly, than anticipated. Despite these difficulties we could deploy and release the device in stable and safe position. Post catheterization, X-Ray of the chest, showed device in good position. The post cath ECG was unchanged. Echocardiography 24 h after the procedure showed device in good position and sandwiching the rims well with no residual shunt. Conclusion Our case highlights the technical difficulties encountered during device closure of ASD II in a case with situs solitus associated with dextrocardia. It further shows that though it is doable, but attention should be paid to the abnormal orientation of the interatrial septum and guidance by hand injections as well as the pressure tracing are occasionally more important than TEE. To the best of our knowledge, this is the first report of a case of successful ASD device closure in a patient with situs solitus and dextrocardia.