A Rare Treatable Cause of Cardiomyopathy: Primary Carnitine Deficiency.

Introduction: Primary carnitine deficiency (PCD) is a rare autosomal recessive disorder caused by loss of function mutations in the solute carrier family 22 member 5 (SLC22A5) gene that encodes a high-affinity sodium-ion-dependent organic cation transporter protein (OCTN2). Carnitine deficiency can result in acute metabolic decompensation or, in a more insidious presentation, cardiomyopathy. Cardiomyopathy associated with PCD often presents with life-threatening heart failure. This presentation also usually includes skeletal muscle myopathy. Early recognition of this disorder and treatment with carnitine can avoid life-threatening complications related to cardiomyopathy. Case Presentation: Herein, we present a 10-month-old male patient with PCD, which was diagnosed while investigating the etiology of dilated cardiomyopathy and confirmed by molecular genetic analysis. Conclusion: Homozygous c.254_265 insGGCTCGCCACC (p.I89Gfs) pathogenic variant of the SLC22A5 gene was detected. With oral L-carnitine supplementation, the free carnitine level increased up to 14 µmol/L and the symptoms disappeared. LVEF increased by 45-70%. We would like to emphasize that this problem is a combination of the metabolic decompensation and the cardiac phenotypes, which are usually separated to either phenotype.

The influence of maternal COVID-19 on cardiac functions: From fetal life to infancy.

OBJECTIVE: To evaluate both short and mid-term effects of maternal COVID-19 on cardiac functions of fetuses and children. METHODS: The present case-control study was conducted on 36 pregnant women who had COVID-19 infection in the second trimester of pregnancy and 30 pregnant women as healthy controls. Fetal, neonatal, and infant cardiac functions were compared between the groups. Assessment of fetal cardiac functions were performed in the last trimester of the pregnancy at least 6 weeks after the recovery of infection. The first postnatal echo was performed within the first 2 weeks and the follow-up (second) echo was performed in the 6-8 weeks of life. RESULTS: The demographic data were similar between groups. Interventricular septum and left ventricular posterior wall end-diastolic dimensions were significantly higher in the study group in both fetal, neonatal, and infant periods. Impaired diastolic functions of right and left ventricles were detected and myocardial performance indexes with tissue doppler imaging of both lateral walls and septum were significantly higher than controls at all periods. CONCLUSION: Maternal COVID-19 seems to have a global impact on the cardiac functions of babies in the short and mid-term periods after maternal recovery.

Transient second-degree high-grade atrioventricular block caused by myocarditis.

Myocarditis is an inflammatory disease of the heart muscle that most commonly occurs after infectious diseases in childhood. The clinical picture of acute myocarditis ranges from asymptomatic infection to fulminant heart failure and sudden death (1). Most of the patients may present with nonspecific symptoms such as respiratory distress, chest pain, nausea, and vomiting (2). While rhythm abnormalities such as ventricular and supraventricular rhythm disorders can be observed in these patients, various degrees of atrioventricular blocks may rarely develop (3). In this article, we aimed to present a patient who developed second-degree, high-grade atrioventricular block after myocarditis and recovered completely after treatment.

Assessment of Myocardial Mechanics in Acute Rheumatic Fever Using Speckle-Tracking Echocardiography.

BACKGROUND: This study aims to evaluate the role of speckle-tracking echocardiography to identify myocardial deformation in acute rheumatic fever. METHODS: Twenty-seven patients and 27 healthy children were prospectively evaluated. The patient group was divided into 2 subgroups based on echocardiographic findings, with or without carditis. The left ventricular global longitudinal strain and strain rate, left ventricular global circumferential strain and strain rate, and right ventricular global lon-gitudinal strain and strain rate were assessed by speckle-tracking echocardiography. RESULTS: In the acute phase of the disease, all values except the right ventricular global longitudinal strain were found to be significantly below the control group in the patient cohort. No significant difference was found between the patients grouped as carditis and non-carditis in the acute period. Comparison of the acute period with the post-treatment period revealed a significant increase in all strain values of the patients with carditis and significant increases observed in all values except left ventricular global longitudinal strain rate, left ventricular global circumferential strain rate, and right ventricular global longitudinal strain rate values in patients without carditis. Apart from the right ventricular global longitudinal strain rate, which was significantly lower in the non-carditis group compared to the control group, there was no significant difference in strain values between the patient and control groups following treatment. CONCLUSION: In the present study, we found that all patients, including patients in whom no valvular involvement was detected by echocardiography in the acute phase of acute rheumatic fever, had a lower right and left ventricular strain and strain rate measurements and that these findings improved after treatment, suggesting that strain echocardiography may be a helpful diagnostic method, especially in patients without valvular involvement.

The Effects of Vitamin D on Myocardial Function Demonstrated by Speckle-Tracking Echocardiography in Children with Beta Thalassemia.

OBJECTIVE: Beta thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is a feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle. The aim of our study is to evaluate the association between vitamin D deficiency and deformation of the left ventricular myocardium measured by speckle-tracking echocardiography in children with thalassemia major. METHODS: In this prospective study, 33 thalassemic patients with vitamin D deficiency were enrolled. Cardiac magnetic resonance T2* value, conventional echocardiography, and speckle tracking, and also left ventricular longitudinal and circumferential strain values were measured. Myocardial functions of the patients with vitamin D deficiency or insufficiency were evaluated by speckle-tracking echocardiography before and after vitamin D replacement. RESULTS: The mean age of the patients was 15.4 ± 3.09 years. Vitamin D level was deficient in 30 (90%) and insufficient in 3 (10%) of them. Speckle-tracking analysis showed a significantly decreased absolute value of the left ventricular global longitudinal strain before vitamin D replacement. A significant improvement in the global longitudinal strain was detected after vitamin D replacement (P < 0.05). A statistically significant increase was observed in parameters showing left ventricular systolic and diastolic functions after vitamin D replacement. CONCLUSION: Vitamin D deficiency is frequently observed and causes decreased contractility in thalassemic patients. In our study, we observed that our patients' cardiac functions had improved after vitamin D replacement therapy.

Changing face of acute rheumatic fever in childhood and our clinical results.

OBJECTIVE: This study aims to evaluate the demographic and clinical findings of acute rheumatic fever (ARF) patients followed up in our clinic, their responses to treatment, and prognoses and to determine the clinical utility of echocardiography (ECHO) in the diagnosis of ARF. METHODS: We retrospectively evaluated the data of 160 patients with ARF (6-17, mean 11.7±2.3 years, F/M: 88/72) that was diagnosed according to the Jones criteria and followed up in the pediatric cardiology clinic between January 2010 and January 2017. RESULTS: About 29.4% (n=47) of 104 patients with rheumatic heart disease (RHD) had subclinical carditis. It was observed that subclinical carditis was most common in patients with polyarthralgia (52.2%); in contrast, clinical carditis was most commonly observed together with chorea (39%) and polyarthritis (37.1%). It was found that 60% (n=96) of the patients with rheumatic fever were between the ages of 10-13 and 31.3% (n=50) presented arthralgia most frequently in the winter months. The most common concomitant major symptoms were carditis + arthritis (35%) and carditis + chorea (19.4%). In patients with carditis, the most affected valves were mitral (63.8%) and aortic (50.6%) valves, respectively. The prevalence of monoarthritis, polyarthralgia, and subclinical carditis increased in cases diagnosed during and after 2015. The cardiac valve involvement findings of 71 of 104 patients (68.2%) with carditis improved during the approximately 7 years of follow-up. The regression of heart valve symptoms was significantly higher in patients with clinical carditis and those that complied with prophylaxis compared to patients with subclinical carditis and those that did not comply with prophylaxis. CONCLUSION: We conclude that ECHO results should be included in the diagnostic criteria of ARF, and that subclinical carditis is associated with a risk of developing permanent RHD. Secondary prophylaxis non-compliance is significantly associated with recurrent ARF, and early prophylaxis can reduce the prevalence of RHD in adults and potential associated complications.

Early outcomes of the treatment of aortic coarctation with BeGraft aortic stent in children and young adults.

We report our experience and early outcomes of using the BeGraft aortic stent in children, adolescents, and young adults. BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany) requires a smaller long sheath compared to other covered stents, and it has a low profile and adequate radial power. With these features, it can overcome some limitations in the treatment of coarctation, especially in children. This is a single centre retrospective analysis of 11 implanted BeGraft aortic stents in coarctation of the aorta between July 2020 and November 2021. The eleven stents were successfully implanted in 11 patients (10 males). The median age of the patients was 13.7 years (interquartile range 12-16 years), and the median weight was 43 kg (interquartile range 35-62 kg). In five patients, after the stents were opened completely by the first balloon, they were exchanged with a Z-MED II™ balloon, 1-3 mm larger in diameter, and the stents were redilated. The median catheter-derived systolic peak-to-peak pressure gradient was 23 mm Hg (interquartile range 16-37 mmHg) before the procedure and 3 mm Hg (interquartile range 1-5 mm Hg) after the procedure. Except for the partial femoral artery thrombosis in two patients, no other procedural complications were observed in our study. The median follow-up duration was 5 months (interquartile range 2-12 months). During follow-up, only one patient (9%) had stent narrowing that required dilation. Our initial results and short-term follow-up showed that the BeGraft aortic stent implantation and redilation can be performed effectively, safely, and successfully in the treatment of coarctation of the aorta.

Admission and follow-up cardiac magnetic resonance imaging findings in BNT162b2 Vaccine-Related myocarditis in adolescents.

BACKGROUND: There is limited data on the pattern and severity of myocardial injury in patients with COVID-19 vaccination associated myocarditis. OBJECTIVE: We aimed to define the myocardial damage occurring after BNT162b2 vaccination, raise awareness about adverse reactions developing after vaccination, and determine the patterns and scope of Cardiac magnetic resonance imaging (MRI) findings. PATIENTS/METHODS: A total of 9 patients diagnosed with vaccine-associated myopericarditis were followed up. RESULTS: The mean age of the patient at diagnosis was 15.3 ± 1.0 (range: 14-17) years, and all patients were male. Seven patients presented with myocarditis symptoms after their second vaccine dose, one patient presented with pericarditis symptoms after his first dose, and the other patient presented with myocarditis symptoms after his booster dose. The median time at presenting to the hospital was 3 (range: 2-22) days. Seven (77.7%) patients had abnormal electrocardiography (ECG) findings, and the most prevalent finding was diffuse ST-segment elevation. Initial cardiac MRI results were abnormal in all patients, where 8 (88.8%) patients had late gadolinium enhancement, and 5 (55.5%) had myocardial edoema. Three patients showed local left ventricular wall-motion abnormalities. In their follow-up MRIs 3-6 months later, myocardial edoema was present in 2 (28.5%) patients, while late gadolinium enhancement was present in all patients (7/7, 100%, 2 patients did not have control MRI time). Hypokinetic segments were still present in one of the 3 patients. No negative cardiac events were observed in the short-term follow-up of any patient. CONCLUSION: Further follow-up evaluation and larger multicenter studies are needed to determine the clinical significance of persistent cardiac MRI abnormalities.

Mid-cavitary hypertrophy after myocarditis in a patient with operated medulloblastoma.

A 16-month-old girl was referred for tachycardia and upper respiratory tract infection. Echocardiographic examination revealed pericardial effusion, mild mitral regurgitation, and left ventricle systolic dysfunction. Patient was positive for Parainfluenza type 4 virus. Her laboratory tests revealed increased troponin I level. The patient was treated with intravenous immunoglobulin considering acute viral myopericarditis. Two weeks after treatment, midventricular hypertrophy was detected.

Tetralogy of Fallot with double aortic arch and aortopulmonary window: a very rare trifecta.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

Incidental detection of accessory mitral valve in adolescent with atrial septal defect: Diagnosis with 3D transesophageal echocardiography and excision with transaortic method.

BACKGROUND: Accessory mitral valve tissue (AMVT) is an extremely rare causes left ventricular outflow tract (LVOT) obstruction and is usually incidentally detected in childhood. It is often associated with other cardiac and vascular congenital malformations. CASE PRESENTATION: In this case, we present a 15-year-old girl was diagnosed with AMVT by transesophageal echocardiography, resulting in LVOT obstruction during systole. Interestingly enough, the patient's accessory mitral valve remained undetected for years until he became symptomatic for wide ASD. Successful closure of the ASD with resection of the AMVT was performed with a transaortic approach. The patient was hemodynamically stable postoperatively. There were no abnormalities in the mitral valves and LVOT. CONCLUSION: It was also unusual to see AMVT with ASD instead of other frequently associated other congenital anomalies. Accessory mitral valve should be considered a rare but important cause of left ventricular outflow tract obstruction in childhood.

Successful occlusion of a feeding artery with Amplatzer Piccolo Occluder in a patient diagnosed with Scimitar syndrome.

Scimitar syndrome is a congenital anomaly in which some or all of right pulmonary veins drain into inferior caval vein. It is associated with anomalous systemic arteries arising from descending aorta supplying to right lung. Transcatheter embolisation of this artery prevents complications. We present a 2.5-year-old girl in which anomalous artery was embolised using Amplatzer PiccoloTM Occluder, for first time.

Echocardiographic Findings and Correlation with Laboratory Values in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19.

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p < 0.001), E/A ratio was significantly lower, and averaged E/e' ratio was significantly higher (median 1.5 vs. 1.8, p < 0.05; 8.9 vs. 6.3, p < 0.001 respectively) in patients compared to control. Patients were divided into 2 groups according to 3D LV ejection fraction (LVEF) on the echo-1: Group 1; LVEF < 55%, 26 patients, and group 2; LVEF ≥ 55%, 24 patients. E/e' ratio was significantly higher in group 1 than group 2 and control at discharge (median 7.4 vs. 6.9, p = 0.005; 7.4 vs. 6.3, p < 0.001 respectively). Coronary ectasia was detected in 2 patients (z score: 2.53, 2.6 in the right coronary artery), and resolved at discharge. Compared with group 2, group 1 had significantly higher troponin-I (median 658 vs. 65 ng/L; p < 0.001), NT-pro BNP (median 14,233 vs. 1824 ng/L; p = 0.001), procalcitonin (median 10.9 vs. 2.1 µg/L; p = 0.009), ferritin (median 1234 vs. 308 µg/L; p = 0.003). The most common findings were ventricular systolic dysfunction recovering during hospitalization, and persisting LV diastolic dysfunction in the reduced LVEF group at discharge. Coronary artery involvement was rare in the acute phase of the disease. Also, in MIS-C patients, the correlation between LV systolic dysfunction and markers of inflammation and cardiac biomarkers should be considered.

Evaluation of early-onset cardiotoxic effects of anthracyclines used during the treatment of childhood acute lymphoblastic leukemia by speckle-tracking echocardiography.

OBJECTIVE: Anthracyclines are widely used in the treatment of acute lymphoblastic leukemia (ALL). However, cardiotoxicity is the most critical side effect that requires dose limitation. It is thought to occur at first exposure, but the clinical presentation may occur years later. In this study, we aimed to determine the time of initial damage and cardiotoxicity that develops in children with ALL. METHODS: In this prospective study, 13 patients with newly diagnosed intermediate-risk precursor B cell ALL treated with the ALL-IC BFM 2009 protocol were included. Conventional echocardiography, tissue Doppler imaging (TDI), and speckle-tracking echocardiography (STE) were performed in all the patients before chemotherapy, after completing the induction phase, and at the end of the reinduction phase. RESULTS: The mean age of the patients was 7.8±4.6 (3.1-16.3) years. Myocardial velocity during systole (Sm) determined by TDI at the interventricular septum significantly decreased during the induction phase. Despite a decrease in STE parameters, a statistically significant reduction was determined in the global longitudinal strain rate at both left and right ventricles at the end of the induction. Nevertheless, a statistically significant increase was observed among the conventional echocardiographic findings in the left ventricular end-diastolic diameter at the end of the reinduction. CONCLUSION: During the treatment of ALL, subclinical anthracycline-associated cardiotoxicity develops in the early stages of treatment. The findings detected by TDI and STE could be missed by conventional echocardiography. We recommend evaluating patients with these newly developed techniques to detect subclinical cardiotoxicity at an early stage and starting appropriate therapy on time.

Low serum 25-Hydroxy (OH) vitamin D levels are associated with increased arterial stiffness in healthy children: An echocardiographic study from Turkey.

OBJECTIVE: Arterial stiffness refers to arterial wall rigidity, particularly in central vessels, and is an independent predictor of cardiovascular disease in many chronic diseases. 25-Hydroxy (OH) vitamin D has beneficial effects on blood pressure, vascular endothelial function, and arterial stiffness; most importantly, its deficiency is common worldwide. Therefore, we aimed to elucidate the role of 25-OH vitamin D deficiency in arterial stiffness development and its relationship with arterial stiffness in healthy children. METHODS: This study included 80 patients with low levels of 25-OH vitamin D and 40 healthy control subjects. The study participants were then divided into three groups: group 1 consisted of patients with a deficient 25-OH D level of < 19.9 ng/ml, group 2 with an insufficient 25-OH D level between 20 and 29.9 ng/ml; group 3 were considered control group with a sufficient serum 25-OH vitamin D level of ≥30 ng/ml. Aortic strain, distensibility, stiffness index, and standard left ventricular measurements were calculated using M-mode echocardiographic data. RESULTS: Left ventricular mass index (LVMI) and inter-ventricular septal diastolic thickness (IVST) appeared to increase in group 1 compared to groups 2 and 3. Aortic strain and distensibility were significantly decreased in group 1, whereas aortic stiffness index and elastic modulus were significantly increased. The aortic stiffness index was negatively correlated with serum 25-OH vitamin D levels; however, aortic strain, aortic distensibility, and LVMI were positively correlated. CONCLUSIONS: Our study results revealed a significant relationship between 25-OH vitamin D levels indicative of a deficiency and aortic stiffness. Hence, we suggest that arterial stiffness may also occur in healthy children with a 25-OH vitamin D deficiency. Future in-depth studies are needed to understand the exact mechanisms underlying the aortic stiffness development associated with 25-OH vitamin D deficiency.

Premature cardiac beats in children with structurally normal heart: autonomic dysregulation.

BACKGROUND: Premature ventricular contractions and supraventricular contractions are common rhythm disorders requiring comprehensive investigation in children. The aim of the study was to evaluate the heart rate variability (HRV) in premature ventricular contractions (PVCs) and supraventricular contractions (PSVCs) in children. METHODS: The study compared the characteristics of HRV in 175 children with PVCs and 160 children with PSVCs who underwent 24-h Holter monitoring, with 101 healthy children. RESULTS: Significant differences were found between standard deviation of all normal RR intervals (SDNN), standard deviation of average RR intervals in all 5 min segments of registration (SDANN), root mean square of the successive differences (rMSSD), and the proportion of NN50 divided by total number of NNs (pNN50) values of the patient and control groups. The PVCs group had a significantly lower high frequency (HF) and higher low frequency (LF)/HF ratio and the PSVCs group had a significantly higher LF and higher LF/HF ratio compared to the control group. No significant correlation existed between frequency and the SDNN index, rMSSD, and pNN50 values of the PVCs group. The receiver operating characteristics analysis showed a significantly changed LF/HF ratio when premature contractions exceeded 60 beats per hour in children with PVCs. CONCLUSIONS: This study indicated an increased sympathetic tone and a significantly decreased vagal tone in children with PVCs and PSVCs. HRV can be used increasingly as a non-invasive method in the follow-up of children with premature cardiac contractions.

Algorithm for the diagnosis and management of the multisystem inflammatory syndrome in children associated with COVID-19.

OBJECTIVE: Although the initial reports of COVID-19 cases in children described that children were largely protected from severe manifestations, clusters of paediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease, toxic shock syndrome and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Our objective was to develop a protocol for the evaluation, treatment and follow-up of patients with MIS-C. METHODS: The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of paediatric critical care, cardiology, rheumatology, surgery, gastroenterology, haematology, immunology, infectious disease and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback. CONCLUSION: Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a paediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients' phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.

Investigation of the prevalence of cardiovascular risk factors in obese patients diagnosed with metabolic syndrome in childhood and examination of left ventricular function by echocardiography.

OBJECTIVES: The objective of this study is to investigate the cardiovascular risk factors associated with metabolic syndrome (MetS), which is increasingly becoming prevalent in childhood obesity. METHODS: A total of 113 patients, 76 of whom were between the ages of 10 and 17 (mean age: 14.5 ± 1.8 years) and diagnosed with obesity (30 non-MetS and 46 MetS using IDF) and 37 of whom constituted the control group, participated in the study. Echocardiographic examination and atherogenicity parameters (Atherogenic index of plasma [AIP: logTG/HDL], total cholesterol/HDL, and TG/HDL ratio and non-HDL) were evaluated. RESULTS: The most common component accompanying obese MetS was found to be hypertension and low HDL. While obesity duration, body mass index (BMI), blood pressure, fasting insulin, insulin resistance, atherogenicity parameters were determined to be significantly higher in the obese-MetS group. Echocardiography showed that while the thickness, volume, and diameter of LV end-diastolic wall, left ventricular mass (LVM), LVM index (LVMI g/m2) and relative wall thickness (RWT) were significantly high in the MetS group, however, mitral E/A ratio was significantly lower (p<0.05). Change in LV geometry consistent with concentric remodeling (increased RWT, normal LVMI) was visible in obese groups. LVM were positively significantly related to BMI, waist circumference, insulin resistance, blood pressure, LDL level, and negative to mitral E/A ratio. In the obese-MetS group, LVMI was positively correlated to office systolic BP, left atrium end-diastolic volume/index. CONCLUSIONS: LVMI and atherogenicity parameters that were found to be significantly higher in obese MetS exhibit increased cardiovascular risk in childhood.

Malonyl coenzyme A decarboxylase deficiency with a novel mutation.

Malonyl-CoA, a product of acetyl-CoA carboxylase is a metabolic intermediate in lipogenic tissues that include liver and adipose tissue, where it is involved in the de novo fatty acid synthesis and elongation. Malonyl-CoA decarboxylase (MLYCD, E.C.4.1.1.9), a 55-kDa enzyme catalyses the conversion of malonyl-CoA to acetyl-CoA and carbon dioxide, thus providing a route for disposal of malonyl-CoA from mitochondria and peroxisomes, whereas in the cytosol, the malonyl-CoA pool is regulated by the balance of MLYCD and acetyl-CoA carboxylase activities. So far, 34 cases with different MLYCD gene defects comprising point mutations, stop codons, and frameshift mutations have been reported in the literature. Here, we describe the follow-up of a patient affected by malonic aciduria upon neonatal onset. Molecular analysis showed novel homozygous mutations in the MLYCD gene. Our findings expand the number of reported cases and add a novel variant to the repertoire of MLYCD mutations.

A rare association of left pulmonary artery sling with Scimitar syndrome: Recurrent wheezing and respiratory distress in a pediatric patient.

Congenital anomalies of the heart and great vessels may lead to localized recurrent pulmonary infections through different mechanisms. Pulmonary artery sling (left pulmonary artery originating from the right pulmonary artery) and Scimitar syndrome are rare causes of wheezing in infants. An 18-month-old female infant with left pulmonary artery sling, Scimitar syndrome, and an anomalous connection of left pulmonary veins to the left atrium was admitted to our clinic. She successfully underwent transcatheter embolization using the Vascular Plug-II on the anomalous systemic arterial supply and repair of pulmonary arterial sling and Scimitar anomaly via a median sternotomy. In conclusion, diagnosis of left pulmonary arterial sling accompanied by abnormalities can be missed in some cases using echocardiography. It may be necessary to conduct more advanced imaging methods before deciding the treatment method to be performed in these patients.