Balloon-expandable stents for native coarctation of the aorta in children and adolescents.

Although balloon-expandable stent implantation for native aortic coarctation is a preferred method in the adolescent age group, there are insufficient data about indications for and the efficacy of the procedure in a younger age group. The aim of this study was to compare and evaluate the data of young pediatric and adolescent patients who underwent balloon-expandable stent implantation because of native aortic coarctation. The retrospective analysis included the demographic characteristics and data related to the procedure and follow-up of patients who underwent stent implantation for native aortic coarctation between August 2010 and November 2017. Patients with re-coarctation were excluded from the study. The patients were separated into 2 groups as the adolescent group (Group I: 10-18 years) and the pediatric group (Group II: ≤ 9.9 years). Group-I comprised of 18 patients and Group-II, 32 patients. Covered stent was implanted to 32 (73%) patients and uncovered stent to 12 (27%) patients. The procedural success rate was 100%. Following stent implantation, peak systolic gradient decreased significantly in both groups (P < .0001) (Group-I: from 35.9 ±â€…16.6 mm Hg-2.2 ±â€…3.4 mm Hg, Group II: from 34 ±â€…13.3 mm Hg-3 ±â€…4.09 mm Hg). Complications developed in 3 patients, and all in Group I. Femoral hematoma developed in 1 patient, balloon rupture occurred during the procedure in 1 patient, and there was temporary loss of pulse in 1 patient. All the complications were treated successfully. All the patients were taking anti-hypertensive drugs before intervention and during the mean 23-month follow-up period (range, 2-84 months), hypertension recovered in 35 (80%) patients and drugs were terminated. Stent implantation for aortic coarctation in the pediatric age group may provide pleasing results, reducing the coarctation gradient, providing effective dilatation in the lesion area and eliminating hypertension.

A rare cause of abnormal pulmonary venous drainage: septum primum malposition.

OBJECTIVES: This study aimed to evaluate the clinical features of patients with septum primum malposition, imaging tools used for diagnosis, and their effects on the surgical approach. MATERIALS AND METHODS: Patients diagnosed with septum primum malposition in our paediatric cardiac centre between 1 January, 2015 and 1 January, 2019 were included in the study. In all patients, the age, reason for admission, transthoracic echocardiography, cardiac multidetector CT angiography findings, and subsequent surgical data were evaluated. RESULTS: Fifteen patients were diagnosed with septum primum malposition during the study period. The median age was 12 months (2 months-10 years). Six patients were left isomeric, and the rest were situs solitus; 80% of the patients (n = 12) had additional secundum atrial septal defect. There was cardiac pathology in 46% of the patients (n = 7) in addition to the abnormal pulmonary venous drainage, ventricular septal defect (n = 3), left ventricularhypoplasia (n = 2), cortriatriatum sinister (n = 2), double outlet right ventricle (n = 1), and atrioventricular septal defect (n = 1). There was bilateral superior caval vein in three patients, right-sided superior caval vein in 11 patients, and left-sided superior caval vein in one patient. All three patients with total abnormal pulmonary venous drainage were left atrial isomeric. There were differences between the results of transthoracic echocardiography and CT angiographies in two patients. The surgical strategy was changed in three patients after the preoperative diagnosis of septum primum malposition. CONCLUSION: Septum primum malposition should be kept in mind during the imaging of complex CHDs specifically during the segmental analysis of the pathologies with heterotaxy syndromes; it should be differentiated from other aetiologies of abnormal pulmonary venous drainage as accurate diagnosis would facilitate the ideal surgery in these complex pathologies requiring a detailed preoperative preparation.

The assessment of patients with situs inversus and corrected transposition of the great arteries.

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs. OBJECTIVES: The purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail. METHODS: The records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded. RESULTS: Twenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis. CONCLUSION: SI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.

A rare association: first degree AV block and long QT syndrome.

The coexistence of long QT syndrome with 2:1 or complete atrioventricular blocks has been reported in the literature, but, to the best of our knowledge, this is the first pediatric case of long QT syndrome coexisting with first-degree atrioventricular blocks( 1 - 3 ).

Arrhythmia during diagnostic cardiac catheterization in pediatric patients with congenital heart disease.

OBJECTIVE: Diagnostic and interventional cardiac catheterization procedures for congenital heart diseases (CHD) are becoming increasingly more popular, and arrhythmia is a well-known complication. This study was an evaluation of the incidence and causative agents of arrhythmia and the subsequent treatment strategies applied during cardiac catheterization. METHODS: The catheterization data of all of the patients who underwent diagnostic cardiac catheterization for CHD between January 2012 and 2018 at a single center were examined retrospectively. RESULTS: A total of 1316 children underwent diagnostic cardiac catheterization due to CHD. The median age and body weight was 18 months (6 days-21 years) and 9.9 kg (2.2-135 kg), respectively. Patients with ventricular septal defect (281 patients) and those with tetralogy of Fallot (257 patients) represented 2 major groups of the study population. In 93 (7%) patients, arrhythmia developed during cardiac catheterization. Among them, there were 58 (62%) cases of bradyarrhythmia and 35 (38%) cases of tachyarrhythmia. Arrhythmia was classified as low, high, or major, according to the adverse event severity score; the rates were 2.7%, 4.3%, and 1.2%, respectively. In 36 (39%) patients, there was no need for therapy, whereas 57 (61%) required treatment to eliminate the arrhythmia. Treatment modalities included catheter manipulation in 15, pharmacological therapy in 24, and cardioversion in 3 patients. Eleven patients required cardiopulmonary resuscitation. Temporary pacemaker implantation was required in 2 patients, while 2 others underwent permanent pacemaker implantation secondary to catheterization-related arrhythmia. There were no cases of mortality secondary to catheterization-related arrhythmia. CONCLUSION: Diagnostic cardiac catheterization in CHD may result in various types of cardiac arrhythmias. The proper management of arrhythmias may reduce morbidity and mortality related to cardiac catheterization.

Cardiac Catheterization in the Early Post-Operative Period after Congenital Heart Surgery.

BACKGROUND: The number of diagnostic and interventional cardiac catheterization procedures are increasing in the post-operative period of congenital heart diseases (CHD). The aim of this study was to evaluate data of patients who underwent cardiac catheterization in the early post-operative period after congenital heart surgery (CHS). METHODS: We retrospectively evaluated the data of patients who underwent cardiac catheterization within 30 days after CHS. RESULTS: Between 2010 and 2016 in our hospital, 2584 children had operations, and 2911 children underwent cardiac catheterization due to CHD. Cardiac catheterization was performed in 50 (1.9% of the surgeries) of these patients during the early post-operative period. Twenty-nine (58%) of the patients were males. The median age was 7.5 months (range: 15 days-12.5 years), and the median body weight was 6 kg (range: 3-35 kg). Twenty-eight (56%) of the patients had two-ventricle, and 22 (44%) had single ventricle physiology. The median RACHS-1 score was 3 (range: 1-6). Cardiac catheterization was performed under extracorporeal membrane oxygenation (ECMO) support in 16 of the patients. Twenty-four (48%) patients underwent diagnostic catheterization, while 26 (52%) had interventional procedures. Fifteen (30%) patients had a reoperation due to anatomic problems identified during catheterization. Major complications developed in 4 (8%) patients. There was no cases of procedural mortality due to catheterization. CONCLUSIONS: Cardiac catheterization should be performed in post-operative cardiac patients without hesitation, even under ECMO, if significant hemodynamic or clinical problems cannot be identified clearly by other non- interventional diagnostic techniques.

Rhythm disturbances and treatment strategies in children with congenitally corrected transposition of the great arteries.

BACKGROUND: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies. PATIENTS AND METHODS: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared. RESULTS: Group I (n = 53, 82%) consisted of patients with significant associated lesions, and Group II (n = 12, 18%) consisted of those with minor or no associated lesions (isolated ccTGA). Rhythm abnormalities were diagnosed in 22 (34%) of the patients based on initial ECG findings and Holter ECG recordings. Eleven (17%) of these patients had atrioventricular (AV) block of different degrees, and the other 11 (17%) had supraventricular arrhythmia (SVA). The median follow-up was 49 months (range, 9-89 months), and the rhythm remained normal in 26 (42%) of the patients. Three patients died on follow-up. Of 40 patients with normal initial findings, nine required pacemaker implantation due to complete heart block, and SVA developed in seven patients on follow-up. No ventricular tachycardia was seen initially or on follow-up. Ablation was performed in four patients. During the follow-up period, pacemakers were implanted in 12 (23%) of patients in Group I and 4 (33%) of patients in Group II due to complete heart block. Cardiac resynchronization therapy (CRT) was performed in four patients due to systemic ventricular dysfunction. Notably, all four of these patients had a pacemaker implanted postoperatively.

Analysis of congenital heart surgery results: A comparison of four risk scoring systems.

BACKGROUND: This study aims to evaluate the surgical results of our clinic according to presumption systems of Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity score, Aristotle Comprehensive Complexity score, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories and to compare the efficiency of these systems in predicting morbidity and mortality. METHODS: In the study, classification and the risk scoring were performed with the four different systems for 1,950 patients (1,038 males, 912 females; mean age 5.5 months; range, 1 day to 18 years) who were administered congenital heart surgery between 1 October 2012 and 31 December 2016. The hospital mortality and morbidity were calculated for each category from the four models. The discriminatory ability of the models was determined by calculating the area under the receiver operating characteristic curve and the receiver operating characteristic curves of the four models were compared. RESULTS: Median weight of the patients was 7.2 kg (range, 1.8-80 kg). Among the patients, 53% were males and 47.5% were younger than one year of age. Of totally 1,950 operations, mortality was observed in 149 (7.6%) and morbidity was observed in 541 (27.7%). Areas under the receiver operating characteristic curve for mortality were 0.803, 0.795, 0.729, and 0.712 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Aristotle Comprehensive Complexity, Risk Adjustment in Congenital Heart Surgery, and Aristotle Basic Complexity scores, respectively. Areas under the receiver operating characteristic curve for morbidity were 0.732, 0.731, 0.730, and 0.685 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Comprehensive Complexity, and Aristotle Basic Complexity scores, respectively. CONCLUSION: Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity, and Aristotle Comprehensive Complexity score systems were effective in predicting the morbidities and mortalities of patients who underwent congenital heart surgery and evaluating the performance of the surgical centers. Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories were on the forefront due to high feasibility and performance. Aristotle Basic Complexity score system had the lowest performance. Combinations of systems will provide the most benefit during evaluation of results.

Sustained tachyarrhythmia in children younger than 1 year of age: Six year single-center experience.

BACKGROUND: The aim of this study was to evaluate the results of non-operational sustained tachyarrhythmia in patients <1 year of age at the present center. METHODS: Between November 2010 and November 2016, the demographic characteristics, type and localization of the tachyarrhythmia, echocardiographic findings, and medical and/or ablation therapy for patients <1 year of age with sustained tachyarrhythmia were evaluated. RESULTS: Of 99 patients, 91 had sustained supraventricular tachycardia, and eight had sustained ventricular tachycardia. The median age was 30 days (range, 1-350 days), and the median weight was 4.2 kg (range, 2-13 kg). The common symptoms were palpitation and restlessness (n = 49, 49.5%), or the tachycardia was detected during routine inspection (n = 25, 25.3%) or fetal echocardiography (n = 11, 11.1%). Nineteen individuals (19%) had left ventricular (LV) dysfunction on first echocardiography. Twenty individuals had congenital heart disease. Common diagnoses were Wolff-Parkinson-White syndrome (n = 27), focal atrial tachycardia (n = 10), permanent junctional reciprocating tachycardia(n = 6), and atrial flutter (n = 6). Seventeen patients underwent medical therapy combined with cardioversion. The most commonly used abortive and acute therapy agents were adenosine, esmolol, and amiodarone. The most common combination therapy was propranolol and amiodarone, followed by a propranolol and propafenone combination. Nine individuals were treated with catheter ablation due to either resistance to medical therapy or LV dysfunction. CONCLUSIONS: Tachyarrhythmias in children <1 year of age are mostly caused by accessory pathways and require multidrug therapy. Catheter ablation is an effective alternative therapy in the case of resistance to medical therapy and/or LV dysfunction.

Cardiac catheterization procedures in pediatric patients undergoing extracorporeal membrane oxygenation cardiac catheterization, ECMO.

OBJECTIVE: Extracorporeal membrane oxygenation (ECMO) is a lifesaving intervention for pediatric patients with respiratory and/or cardiovascular failure. In this study, we evaluated the cardiac catheterization results of pediatric patients on ECMO support. METHODS: Between January 2012 and October 2016, 98 patients (5.2% of all surgery patients) needed ECMO support during perioperative cardiac surgery. We retrospectively reviewed the clinical data of 16 patients who underwent cardiac catheterization under ECMO support. RESULTS: The median age at catheterization was 6.5 months (range, 3.3-60 months), and the median weight was 6.0 kg (range, 3.7-16 kg ). Eight of the catheterizations were diagnostic, and the remaining eight were interventional. Five out of these eight patients underwent surgical palliation after diagnostic catheterization. Right pulmonary artery (RPA) stenting, right ventricular outflow tract (RVOT) stenting, combined left pulmonary artery (LPA) and RVOT stenting, combined LPA and modified Blalock-Taussig shunt stenting, bilateral pulmonary artery balloon angioplasty, and bilateral pulmonary artery stenting were each performed once, whereas LPA stenting was performed in two different patients. In one patient undergoing RVOT stenting, a complete atrioventricular block developed, resulting in hypotension; however, this was overcome with an ECMO flow increase. In another patient, the ECMO tubing disconnected from the arterial line. Minor vascular complications were seen in three patients. Twelve patients (75%) were successfully weaned from ECMO after the procedure and ten (63%) were discharged. CONCLUSION: Diagnostic and interventional cardiac catheterization can be safely and effectively performed in patients on ECMO. If the patient cannot be weaned from ECMO support, clinicians should consider performing an early angiogram either to treat or clarify the underlying problem.

The Evaluation of Nosocomial Infections in Pediatric Patients with Extracorporeal Membrane Oxygenation Support.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) has become a standard technique over the past few decades in intensive care unit (ICU). OBJECTIVE: A review of pediatric patients who received ECMO support in the pediatric cardiac ICU was conducted to determine the incidence, risk factors and causal organisms related to acquired infections and assess the survival rates of ECMO patients with nosocomial infections. METHODS: Sixty-six patients who received ECMO support in the pediatric cardiac ICU between January 2011 and June 2014 were included in the study. Demographic, echocardiographic, hemodynamic features and surgical procedures were reviewed. RESULTS: Sixty-six patients received a total of 292.5 days of venoarterial ECMO support. Sixty were postoperative patients. Forty-five patients were weaned from ECMO support with an ECMO survival rate of 68.2%. The rate of infection was 116.2/1000 ECMO days. Prolonged ICU stay, duration of ventilation and ECMO were found associated with development of nosocomial infection and only the duration of ECMO was an independent risk factor for nosocomial infections in ECMO patients. CONCLUSION: The correction of the underlying process leading to ECMO support and shortening the length of ECMO duration together with stricter application of ECMO indications would improve the infection incidence and hospital surveillance of the patient group.

The evaluation of nosocomial infections in pediatric patients with extracorporeal membrane oxygenation support

Abstract Introduction: Extracorporeal membrane oxygenation (ECMO) has become a standard technique over the past few decades in intensive care unit (ICU). Objective: A review of pediatric patients who received ECMO support in the pediatric cardiac ICU was conducted to determine the incidence, risk factors and causal organisms related to acquired infections and assess the survival rates of ECMO patients with nosocomial infections. Methods: Sixty-six patients who received ECMO support in the pediatric cardiac ICU between January 2011 and June 2014 were included in the study. Demographic, echocardiographic, hemodynamic features and surgical procedures were reviewed. Results: Sixty-six patients received a total of 292.5 days of venoarterial ECMO support. Sixty were postoperative patients. Forty-five patients were weaned from ECMO support with an ECMO survival rate of 68.2%. The rate of infection was 116.2/1000 ECMO days. Prolonged ICU stay, duration of ventilation and ECMO were found associated with development of nosocomial infection and only the duration of ECMO was an independent risk factor for nosocomial infections in ECMO patients. Conclusion: The correction of the underlying process leading to ECMO support and shortening the length of ECMO duration together with stricter application of ECMO indications would improve the infection incidence and hospital surveillance of the patient group.

Evaluation of Anomalous Coronary Arteries from the Pulmonary Artery.

Objective: This study evaluated clinical and diagnostic findings, treatment methods, and follow-up of cases of anomalous coronary arteries from the pulmonary artery. Methods: The study included all cases diagnosed with anomalous coronary arteries from the pulmonary artery between January 2012 and January 2016. Data from patients' demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, intensive care unit stay, and follow-up were evaluated. Results: The study included 12 patients (8 male, 4 female), 10 with anomalous left coronary artery from the pulmonary artery (ALCAPA) and 2 with anomalous right coronary artery from the pulmonary artery (ARCAPA). Median age at diagnosis was 4 months (range, 1 month - 10 years old) and median weight was 5.5 kg (range, 3-30 kg). The most common complaints were murmur (n=7) and respiratory distress (n=5). In 4 cases, the initial diagnosis was dilated cardiomyopathy. Electrocardiographs were pathologic in all cases. Echocardiographic examination revealed medium to severe mitral valve regurgitation in 4 cases and reduced (< 40%) ejection fraction in 6 patients. Of the 12 patients, 8 underwent direct implantation of the left coronary artery into the aorta, 2 underwent implantation of the right coronary artery into the aorta, and the remaining 2 underwent a Takeuchi procedure. There were no early mortalities. Median hospital stay was 20 days (range, 5-35 days). Median follow-up duration was 18 months (range, 5-36 months), and no cases required further surgery during follow-up. Conclusions: Anomalous coronary arteries from the pulmonary artery can be successfully repaired providing there is early diagnosis and effective, appropriate intensive care unit follow-up. Therefore, coronary artery origins should be evaluated carefully, especially in cases with dilated cardiomyopathies.

Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.

BACKGROUND: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. CASE REPORT: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin's physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block. CONCLUSION: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants.

Evaluation of anomalous coronary arteries from the pulmonary artery

Abstract Objective: This study evaluated clinical and diagnostic findings, treatment methods, and follow-up of cases of anomalous coronary arteries from the pulmonary artery. Methods: The study included all cases diagnosed with anomalous coronary arteries from the pulmonary artery between January 2012 and January 2016. Data from patients’ demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, intensive care unit stay, and follow-up were evaluated. Results: The study included 12 patients (8 male, 4 female), 10 with anomalous left coronary artery from the pulmonary artery (ALCAPA) and 2 with anomalous right coronary artery from the pulmonary artery (ARCAPA). Median age at diagnosis was 4 months (range, 1 month - 10 years old) and median weight was 5.5 kg (range, 3-30 kg). The most common complaints were murmur (n=7) and respiratory distress (n=5). In 4 cases, the initial diagnosis was dilated cardiomyopathy. Electrocardiographs were pathologic in all cases. Echocardiographic examination revealed medium to severe mitral valve regurgitation in 4 cases and reduced (< 40%) ejection fraction in 6 patients. Of the 12 patients, 8 underwent direct implantation of the left coronary artery into the aorta, 2 underwent implantation of the right coronary artery into the aorta, and the remaining 2 underwent a Takeuchi procedure. There were no early mortalities. Median hospital stay was 20 days (range, 5-35 days). Median follow-up duration was 18 months (range, 5-36 months), and no cases required further surgery during follow-up. Conclusions: Anomalous coronary arteries from the pulmonary artery can be successfully repaired providing there is early diagnosis and effective, appropriate intensive care unit follow-up. Therefore, coronary artery origins should be evaluated carefully, especially in cases with dilated cardiomyopathies.

Transesophageal echocardiography experience in thepediatric age group in a tertiary cardiac center.

BACKGROUND/AIM: The aim of this study was to evaluate the transesophageal echocardiography (TEE) findings of pediatric patients in a tertiary center where complex congenital heart surgery and interventional procedures have been performed. MATERIALS AND METHODS: All TEE studies performed between December 2009 and December 2014 were reviewed retrospectively. Patients were divided into 3 groups: perioperative, during interventional procedures, and due to other reasons. Demographic features, transthoracic echocardiography (TTE) reports, TEE reports, change in decision after TEE evaluation, and related complications were recorded. RESULTS: A total of 703 patients who had TEE evaluation were included in the study; 51% were female and 49% were male. The median age was 90 months (2 months to 18 years). TEE was performed perioperatively in 430 patients (61%), during cardiac catheterization-angiography and electrophysiology studies in 181 patients (26%), and due to other reasons in 92 patients (13%). Mismatches between TTE and TEE or changes in decision after TEE evaluation were present in 45 patients (10.4%) who had perioperative TEE, in 10 patients (5.5%) who had TEE during interventional procedures, and 22 patients (24%) who had TEE evaluation due to other reasons. No major complications were detected. CONCLUSION: Information acquired by TEE increases the clarity of future plans for the patient and helps to decrease the mortality and morbidity caused by unnecessary procedures.

A rare pathology: Levoatriocardinal vein.

OBJECTIVE: Levoatriocardinal vein (LACV) is a rare cardiac pathology that represents a connection between the pulmonary venous and cardinal systems. The aim of the present study was to discuss morphological and clinical characteristics, as well as diagnostic methods, of experience with LACV. METHODS: Records of 11 patients (4 male, 7 female; mean age 79±1.83 days; range 1-390 days) diagnosed with LACV between 2010 and 2014 were retrospectively reviewed. Presence of LACV was confirmed with echocardiography. The primary obstructive lesion associated with cardiac defects and the integrity of the interatrial septum was identified in each patient with left-sided obstruction. RESULTS: Mean weight was 4.4±0.4 kg (range: 2-8). Age at presentation was under 1 year in 82% of patients. Nine patients had left-sided obstruction, and 2 had normal intracardiac anatomy and pulmonary venous return. In patients with left-sided obstruction, LACV was initially demonstrated with echocardiographic evaluation, performed in apical 4-chamber, high parasternal, and subcostal views. Atrial septum was restrictive or intact in patients with left-sided obstructions. LACV originated directly from the left atrium in all patients. CONCLUSION: Levoatriocardinal vein is an extremely rare cardiac pathology, presenting almost exclusively in patients with left-sided obstructive lesions. In patients with left-sided obstructions, LACV must be kept in mind. It may also present in patients with normal intracardiac anatomy and pulmonary venous return.