Rare Congenital Heart Disease Research Articles

Complex Adult Congenital Heart Disease: A Rare Case of Dextrocardia with Double-outlet Right Ventricle

Abstract Double-outlet right ventricle (DORV) is a complex and rare congenital heart disease (CHD), which is usually symptomatic early in childhood and thus diagnosed early. Dextrocardia is another rare CHD that can occur either as an isolated cardiac abnormality or may be associated with other complex cardiac defects. However, the co-occurrence of DORV and dextrocardia is rare, especially in adult life. We present a 35-year-old Nigerian lady with situs inversus totalis and complex CHD, which included dextrocardia with multiple congenital intracardiac shunts, DORV occurring with L-malpositioning of the great vessels, atrial septal defect, and ventricular septal defect with partial anomalous pulmonary venous drainage, persistent left superior vena cava as well as severe pulmonary stenosis.

A Case of Late Presentation of Supracardiac Total Anomalous Pulmonary Venous Connection in an Adult

Abstract Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease and their survival into adulthood is even rarer. Here, we present the case of a 26-year-old female who was incidentally diagnosed with a case of supracardiac TAPVC during her pregnancy. All four pulmonary veins were joining to form a common venous confluence which drained into a left-sided vertical vein which drained into the innominate vein.

Laparotomy in a patient with Ebsteins anomaly- from OT to ICU.

Ebstein’s anomaly (EA) is a rare cyanotic congenital heart disease, affecting 1/200,000 live births and accounts for <1% of congenital heart diseases.1 First described by Wilhelm Ebstein in 1866 2 , the anomaly consists of downward displacement of the septal and posterior leaflet of tricuspid valve resulting in atrialization of the right ventricle and tricuspid regurgitation. The resulting functional impairment of right ventricle retard the forward flow of blood through right side of the heart, thereby causing a hypoplastic Right ventricle(RV), or RV outflow tract obstruction. This creates an accessory conduction pathways resulting in tachyarrythmias, pulmonary hypertension, intracardiac shunting. 3 Acute decompensated heart failure and sudden collapse are the most common cause of death.4

Laparotomy in a Patient with Ebsteins Anomaly- From OT to ICU

Ebstein’s anomaly (EA) is a rare cyanotic congenital heart disease, affecting 1/200,000 live births and accounts for <1% of congenital heart diseases [1]. First described by Wilhelm Ebstein in 1866 [2], the anomaly consists of downward displacement of the septal and posterior leaflet of tricuspid valve resulting in atrialization of the right ventricle and tricuspid regurgitation. The resulting functional impairment of right ventricle retard the forward flow of blood through right side of the heart, thereby causing a hypoplastic Right ventricle (RV), or RV outflow tract obstruction. This creates an accessory conduction pathways resulting in tachyarrythmias, pulmonary hypertension, intra-cardiac shunting [3].Acute decompensated heart failure and sudden collapse are the most common cause of death [4].

Cor Triatriatum Sinister Presenting as Cardioembolic Stroke in a Young Woman.

Cor triatriatum sinister is a rare congenital heart disease characterized by an additional fibromuscular membrane in the left atrium. Cardioembolic stroke is a rare complication of cor triatriatum sinister, especially among women. We hereby describe the case of an 18-year-old female patient, without a past medical history, presenting with cardioembolic stroke in the territory of the right posterior cerebral artery. During extensive diagnostic work-up, nonrestrictive cor triatriatum sinister and patent foramen ovale were diagnosed using transthoracic and transesophageal echocardiography. In clinical practice, it is important to identify congenital cardiac defects as potential substrates for cardioembolism in young patients. In our case, cor triatriatum sinister presenting as ischemic stroke was diagnosed, which is an uncommon finding, especially in young females. Determining the optimal management strategy for patients with cor triatriatum sinister complicated by cardioembolic stroke requires a multidisciplinary approach.

Agénésie de la valve pulmonaire avec communication interventriculaire découverte à l'âge adulte suite à une endocardite infectieuse : à propos d'un cas

Absent pulmonary valve syndrome is a rare congenital heart disease. Associated with ventricular septal defect, it is considered a rare variant of Tetralogy of Fallot “Tetralogy of Fallot with absent pulmonary valve syndrome”. It is characterized by its association with aneurysmal pulmonary arteries responsible for airways compression. Survival to adulthood of this unrepaired congenital heart disease is very rare, and the case of the patient we report in this article is added to the rare cases reported in the literature. Clinical tolerance depends on the degree of severity of the malformation and in particular on the importance of the aneurysmal dilation of the pulmonary arteries, thus determining the age of the diagnosis, the severity of symptoms, and the mode of evolution. Diagnosis of Tetralogy of Fallot with absent pulmonary valve syndrome must be established by transthoracic echography. Other investigations can be of capital contribution, such as thoracic computed tomography angiography and cardiac catheterization. The treatment is surgical and includes closure of the ventricular septal defect, relieve right ventricular outflow tract obstruction, and surgical reduction of the aneurysmal pulmonary arteries.

278 A CASE OF ACCIDENTAL DIAGNOSIS OF ALCAPA SYNDROME IN ASYMPTOMATIC CHILD

Abstract A 7-year-old (y.o.) boy was referred to our center for a specialist evaluation due to alteration of the ventricular repolarization in the anterior and lateral lead on the electrocardiogram at medical evaluation for physical activity. The child was totally asymptomatic, with normal growth and unremarkable past medical history. The physical examination revealed only S4 and a minimal apical systolic murmur. Transthoracic echocardiogram demonstrated a mildly dilated left ventricle (LV) and normal left ventricular ejection fraction. Strain pattern of basal-medium segments of lateral wall was reduced. Right coronary artery (RCA) appeared dilatated and the left coronary artery (LCA) showed an inverse flow at Doppler evaluation. Findings were suspicious for ALCAPA diagnosis (Anomalous Left Coronary Artery Arising from the Pulmonary Artery); a rare congenital heart disease that, if not treated, leads to death in 90% of cases during first year of life. Adult type of ALCAPA (adolescence-60 y.o) is even more rare and can cause heart failure due to a dilatative cardiomyopathy, chronic cardiac ischemia and, in 80-90% of cases, sudden cardiac death. We performed a coronary angiography that confirmed the anomalous origin of LCA from the pulmonary artery. The RCA was dominant, enlarged and supplied an important collateral flow for LCA. Coronary angiography is indicated not only for diagnosis confirmation, but also for coronary tree demonstration, especially in adult type of ALCAPA, because of the presence of not rare anomalies or “adaptation” of peripheral coronary circulation. All these features are important positive prognostic factors that enable survival beyond infancy. LCA ostium stenosis or bronchial artery collateral vessels are also common but weren't seen in this case. In adult type of ALCAPA surgery is usually indicated at the time of diagnosis, so a coronary button transfer and pulmonary Valsalva sinus reconstruction with autologous pericardial patch were performed. This is the preferred surgical technique in adult patients as it is the most anatomic correction. Since before hospitalization heart failure therapy with betablocker was introduced and after surgery aspirin therapy was started too. Post-surgery transthoracic echocardiogram highlighted a moderate pericardial effusion without haemodynamic impact and a pleural effusion, so an anti-inflammatory therapy with ibuprofen, cortisone and then colchicine was started. After discharge anti-inflammatory therapy was under-titrated until suspension based on gradual reduction of pericardial and pleural effusion. Patient was always free of symptoms and with good performance status. ALCAPA or Bland–White–Garland syndrome is an uncommon congenital heart disease. The presence of adequate collateral circulation between RCA and LCA allows to survive beyond infancy (adult type). We described a case of early diagnosis of adult type of ALCAPA in asymptomatic child thanks to a careful examination of basal electrocardiogram for evaluation of non-competitive sport practice.

209 A COMMON CASE OF AORTIC STENOSIS IN A UNIQUE QUADRICUSPID ANATOMY: MANAGEMENT IN REAL LIFE

Abstract A 85-year-old lady with severe dyspnea presented in emergency dept. of our institution in Reggio Emilia last July. Typical signs and symptoms of heart failure were associated with high voltage at ECG and echocardiographic findings show a reduced EF (40%) with trans-aortic mean gradient of 42 mmHg, aortic valvular area of 0.8 cm2 and moderate aortic regurgitation. At CT scan analysis, a dismorphic quadricuspid aortic valve (QAV) was detected. This is a rare congenital heart disease generally unrelated to other cardiac abnormalities and usually diagnosed incidentally. Due to QAV stenosis rarity, many considerations in Trans Aortic Valve Implantation (TAVI) in QAV are unclear. Some individuals with QAV develop aortic regurgitation and rarely other cardiovascular complications such as aortic aneurysm. Because of its infrequency, it is difficult to characterize these patients and standardize their management. Tailored therapy is more than ever necessary. Our patient also had bicameral pacemaker for grade II Mobitz 2 atrioventricular blockage. At coronarography angiography mild atheromasia was detected; AngioCT reconstructions in figure 1 panel A and B show the presence of QAV. Severe wall atheromasia along the entire course of the aorta was also detected. A few days later the patient underwent the TAVI procedure. A coronary guidewire was positioned in right coronary artery in order to protect its ostium that was slightly lower and, as known from literature, QAV has a longer leaflet height and a shallower cusp depth. After implantation of a Balloon Expandable Valve, a chimney stenting was also performed. An excellent final result was achieved with a safe deployment and a 90/10 final position (last aortography frame in Figure 2, where both coronary arteries are well visible). Post-procedural echo follow-up showed a well functioning aortic prosthesis (with mean gradient of 9 mmHg). The case presented is particularly interesting mainly for three reasons: Extreme rarity of this condition that can be challenging especially in the elderly people with calcific valve affected by stenosis and regurgitation;Paucity of procedural data in literature on QAV;It is a real life management example with subsequent successful implantation of the BEV and chimney stenting in QAV;

Ebstein's anomaly with pericardial effusion in a 55-year-old lady: A case report

and importance: Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve associated with atrialization of the right ventricle. Most of the cases are diagnosed in childhood but asymptomatic cases may remain undiagnosed and survive until old age. We present a rare case of Ebstein's Anomaly with pericardial effusion which was diagnosed for the first time in her mid-fifties when she developed atrial fibrillation and right heart failure with severe tricuspid regurgitation, which was managed medically. The patient with Ebstein's anomaly can be found even at an older age with variable presentation and the association with pericardial effusion although very rare can present in such patients. Ebstein's anomaly despite being a rare congenital condition can present clinically even beyond the age of fifty without any previous diagnosis and surgical intervention for the condition. It usually presents with features of heart failure and arrhythmia but can also have a rare association like pericardial effusion.

Identification and characterization of novel elastin gene mutations in eleven families with supravalvular aortic stenosis.

Background: Supravalvular aortic stenosis (SVAS) is a rare congenital heart disease affecting approximately 1 in 25,000 live births. In some patients it is accompanied by pulmonary artery stenosis, particularly of pulmonary artery branches. Chronic stenosis can lead to cardiac hypertrophy and even circulatory failure. Familial autosomal dominant SVAS is frequently associated with elastin (ELN) gene mutations, whereas Williams-Beuren syndrome is a complex developmental disorder caused by heterozygous microdeletions of 26-28 genes at 7q11.23, including ELN. Methods: Whole-exome sequencing was performed in 42 individuals from 11 Chinese families with SVAS to identify the pathogenic gene mutations involved. Aortic tissue was obtained for histological analyses, and quantitative reverse-transcription-PCR and western blotting were used to verify the expression of elastin molecules. Results: Five point mutations and six frameshift mutations in the ELN gene were detected in the peripheral blood of all investigated families. Nine were nonsense mutations that result in premature stop codons, and the other two were missense mutations. All variants were heterozygous. Nine of the variants were novel, and have not been included in databases or previously reported. One mutation occurred in individuals from two different families. Reduced elastin protein expression was evident in patients' aortic tissue. Conclusions: The novel mutations of ELN were found to be pathogenic, which confirmed by reduced elastin expression and leads to SVAS. Thus, detailed cardiac testing and genetic counseling are warranted for patients and asymptomatic individuals with these mutations.

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

Few experimental model systems are available for the rare congenital heart diseases of double inlet left ventricle (DILV), a subgroup of univentricular hearts, and excessive trabeculation (ET), or noncompaction. Here, we explore the heart of the axolotl salamander (Ambystoma mexicanum, Shaw 1789) as model system of these diseases. Using micro-echocardiography, we assessed the form and function of the heart of the axolotl, an amphibian, and compared this to human DILV (n = 3). The main finding was that both in the axolotl and DILV, blood flows of disparate oxygen saturation can stay separated in a single ventricle. In the axolotl there is a solitary ventricular inlet and outlet, whereas in DILV there are two separate inlets and outlets. Axolotls had a lower resting heart rate compared to DILV (22 vs. 72 beats per minute), lower ejection fraction (47 vs. 58%), and their oxygen consumption at rest was higher than peak oxygen consumption in DILV (30 vs. 17 ml min−1 kg−1). Concerning the ventricular myocardial organization, histology showed trabeculations in ET (n = 5) are much closer to the normal human setting than to the axolotl setting. We conclude that the axolotl heart resembles some aspects of DILV and ET albeit substantial species differences exist.

Abstract 10439: Assessment of Left Atrial Deformation in Patients With Total Anomalous Pulmonary Venous Connection by Two-Dimensional Speckle-Tracking Echocardiography - A Single-Center Retrospective Cohort Study

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by insufficient left ventricle growth and diastolic dysfunctions. We hypothesized that reduced blood flow into the left heart in the uterus could also affect left atrium (LA) growth, leading to anomalies after birth. We compared the age-related changes in LA parameters detected using two-dimensional speckle-tracking echocardiography (2DSTE) in patients with TAPVC. Method: The LA datasets of 28 neonates with isolated TAPVC were analyzed before surgery and during follow-ups and compared with those of age-matched healthy controls (January 2009–2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) functions. LA pressure was determined by performing catheterization. Results: Compared to the control group, the TAPVC group had significantly smaller LA maximum size preoperatively and normal and larger time-dependent increase in the LA maximum and minimum size, respectively. All 2DSTE-determined LA functions showed significant reductions at all time points in the TAPVC group compared to those in the control group (median εR, εCD, and εCT; before surgery: 17.0 vs. 26.0, 12.9 vs. 15.9, and 6.3 vs. 10.4; follow up at 1-2 years: 30.0 vs. 45.7, 23.2 vs. 29.6, and 6.1 vs. 16.3; follow up at 5-7 years: 31.2 vs. 43.1, 25.0 vs. 31.2, and 5.2 vs. 10.8, respectively; p < .01). However, compared to before surgery, εR and εCD did represent a significantly increased trend at follow-ups (median εR and εCD: 16.8→27.5 and 1.1→23%, respectively). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. Conclusion: This study showed that LA impairments existed even after repair in patients with TAPVC, suggesting that LA function loss mediated by less blood flow persist in childhood.

A case of Ebstein’s anomaly

Ebstein’s anomaly (EA) is a rare congenital heart disease. It has varied clinical presentation depending upon the spectrum of deformation and function of the tricuspid valve and other structures. A comprehensive evaluation by non- invasive modalities is required for optimal decision making regarding the approach to surgical correction of the disease.

Total anomalous pulmonary venous return with mixed drainage and double connection: a rare case report not previously described

Total anomalous pulmonary venous return is a rare cyanotic congenital heart disease in which pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. Given its variability, it is essential to define the pathway and drainage site. We describe a 3-day-old patient with total anomalous pulmonary venous return, in which the four pulmonary veins drained into a common vertical vein that had a double connection at the cardiac (coronary sinus) and infracardiac (portal vein) levels. The echocardiographic diagnosis was confirmed by computed tomography scan. At 1.5 months, she underwent surgery by anastomosis of the venous collector with the left atrium, unroof of the coronary sinus, and clousure of its communication with right atrium. The vertical vein was ligated. There were no complications after 1 year of follow-up. This is an unusual anatomy that has not been described in the literature. In this article, we discuss the embryological origin. Furthermore, we highlight the importance of an accurate diagnosis of the venous pathway and its impact on surgical planning and patient prognosis.

Conservative vs surgical management in ebstein's anomaly: long-term follow-up in a real-life cohort

Abstract Background Ebstein's anomaly (EA) is a rare congenital heart disease that involves the tricuspid valve and the right ventricle, often associating atrial septal defects and atrial arrhythmias. Oligosymptomatic adults affected by this disease usually have good prognosis, while its evolution in symptomatic children is more aggressive. Clinical guidelines provide some recommendations for the former patients regarding surgical indication, but its evidence is scarce and benefit remains unclear. Purpose To compare functional capacity, ventricular function parameters and outcomes between medically and surgically treated patients with EA. Methods Retrospective cohort study of patients with EA followed-up in an adult congenital heart disease reference unit of a tertiary university hospital. Functional status and ventricular size and function parameters at baseline and follow-up, as well as clinical events, were compared between conservative and surgical groups. Results We included 72 patients; mean age was 45.2±15.2 years and 27 (37.5%) were men. Median follow-up was 6.6 (range 0.3–20.3) years. Finally, 18 (25%) patients were operated (12 tricuspid repairs, 5 tricuspid bioprostheses and 1 mechanical tricuspid prostheses). Characteristics and outcomes of conservative and surgical group are shown in Table 1. Comparison of follow-up data for each group are shown in Table 2. Of note, Glenn procedure was done in 6 (33.3%) patients of the surgical group and prosthetic thrombosis was observed in 3 (50%) patients during follow-up. Conclusions Only one fourth of patients with EA in our cohort were managed surgically. Operated patients had worse functional status and larger right ventricles at baseline. At follow-up, surgical patients had more clinical events and worse ventricular function. Exercise test parameters did not change significantly after surgery, whereas there was worsening functional capacity during follow-up in medically-managed patients. Funding Acknowledgement Type of funding sources: None.

Ebstein's anomaly of the tricuspid valve management – the importance of right ventricle assessment

Abstract Background Ebstein's anomaly (EA) is a rare congenital heart disease (CHD) characterized by well known echocardiographic (ECHO) features. The golden standard method for evaluating these patients is cardiac magnetic resonance (CMR) although not easily accessible. The aim of the study was to evaluate the correlation between ECHO and CMR parameters and to determine the optimal parameters for long-term follow-up. Performed was a retrospective cross-section analysis from our registry, which we consider national, as we serve as the only national adult CHD centre. Material and methods Of the 48 patients in the EA national registry, 28 non-operated adults (60% male, median age 39 (18–64) years) with performed CMR were included. Analyzed were ECHO and CMR right ventricular (RV) characteristics, NTproBNP and 6-minute walking distance (6MWD). ECHO parameters were evaluated as follows: 1. morphometric [tricuspid valve septal cusp displacement (mm), long-axis RV end-diastolic dimension (RVEDD,mm), long-axis end-diastolic right to left ventricle diameter ratio (RV/LV)] and 2. functional [tricuspid annular plane systolic excursion (TAPSE,mm), RV fractional area change (FAC,%), RV tissue Doppler myocardial velocity (m/s)]. CMR-derived analyzed paramaters were: ejection fraction (EF,%) and end-diastolic volume (EDV,ml) indexed per body surface area (BSA,m2). According to CMR-derived EDV, patients were divided into groups: 1. normal size RV (55–105ml/m2), 2. dilated RV (>105ml/m2) and 3. small RV (<55ml/m2). Results For ECHO morphometric measurements, Spearman's correlation (R) showed strong, statistically significant correlation of CMR-derived EDV with ECHO-based RV/LV ratio (R=0.6989, P<0.0001); and moderate, statistically significant correlation with ECHO-based RVEDD (R=0.55, P=0.004); No significant correlations between CMR-derived EF and ECHO parameters were found. CMR-derived EF and EDV were independent of the severity of septal leaflet displacement; CMR-derived EF was shown to be conditional to RV EDV, with mean EF in Groups 1/2/3 as follows: 57%/36.5%/25.8% (P=0.0013). CMR-derived Group 3 (small RV) compared to Groups 1 and 2 exhibited worst 6MWD (mean 416m) and highest NTproBNP (mean 1021ng/l) though without statistical significance. Conclusions The assessment of RV in EA seems more critical than the morphology of the tricuspid valve itself. Therefore, as in the context of our results, more emphasis needs to be placed on RV size. In particular, small-capacity RV, as we found, showed strong relation to reduced RV function and some (though not significant) to NTproBNP and 6MWD. CMR-derived EDV and EF are accepted as the gold standard method for RV evaluation. Since we detected significant correlation of CMR with simply gained ECHO parameters (RV/LV ratio and RVEDD), we assume that easily accessible ECHO remains a pertinent method in routine follow-up, able to reflect the complex hemodynamic situation in EA. Funding Acknowledgement Type of funding sources: None.

Outcomes and occurrence of post-operative pulmonary hypertension crisis after late referral truncus arteriosus repair.

BackgroundTruncus arteriosus (TA) is a rare congenital heart disease with a high rate of early mortality. The occurrence of post-operative pulmonary hypertension crisis (PHC), known to be a common and life-threatening complication, increases due to the irreversible development of pulmonary vascular resistance with age. We sought to figure out the risk factors for PHC and describe the surgical outcomes of TA patients with late referral (repair <1 month excluded).Materials and methodsWe retrospectively reviewed patients after TA repair between 2009 and 2021 at Shanghai Children’s Medical Center. The occurrence of PHC was defined according to post-operative Pp/Ps ≥ 1 and clinical manifestations. Risk factors for PHC and mortality were conducted by multivariable analysis.ResultsA total of 98 patients were treated, including 55 males and 43 females. The median age at repair was 121 (69, 245) days. Post-operative PHC occurred in 22 (22.4%) patients with a median age of 186 (122, 293) days. By multivariable analysis, patients with the sum of Z-score of pre-operative bilateral pulmonary artery (PA) diameters (OR: 1.6, 95% CI: 1.2–2.3, P = 0.01) was more likely to experience PHC. Longer CPB duration contributed to early death (OR: 1.0, 95% CI: 1.0–1.0, P = 0.01). Total survival at 10 years was 81.4%. In 4.5 (2.9, 7.5) years of follow-up, twenty-six patients received 30 reinterventions. Valved reconstruction of RVOT most predicted reinterventions (OR: 4.2, 95% CI: 1.4–13.0, P = 0.01).ConclusionSurgical repair of TA patients with late referral has resulted in comparatively favorable early and mid-term outcomes. PHC occurred more commonly in patients with overextended bilateral PA pre-operatively. Meanwhile, valved reconstruction of RVOT would more likely lead to early reintervention.

Double chambered right ventricle with perimembranous defect and severe aortic aneurism: A case in an adult patient

Double-chambered right ventricle (DCRV) is a rare congenital heart disease characterized by the division of the right ventricular

Surgical correction of total anomalous pulmonary venous return in an adult patient

BackgroundTotal anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. Most untreated patients with large atrial septal defects and no pulmonary venous obstruction have pulmonary vascular damage from pulmonary over circulation.Case presentation44-year-old TAPVR patient admitted to our medical center. A snowman-shaped heart, including cardiomegaly and an increase in pulmonary blood flow, was seen in the chest X-ray. A large-sized (around 3 cm) atrial septal defect with dilated right atrium, right ventricle, and pulmonary artery was detected on echocardiography. Heart computed tomography was performed for further evaluation, and supra-cardiac type TAPVR without any obstructive lesion was identified.ConclusionsTAPVR in an adult patient is extremely rare, and this patient was treated successfully with surgical correction and is doing well. A sinus rhythm and mild mitral valve regurgitation have remained during 2.5 years of outpatient follow-up.

Late and Incidental Discovery of an Anomalous Coronary Artery Originating from the Opposite Sinus after Stemi

Coronary artery anomalies (CAAs) comprise a diverse group of congenital malformations with widely varying expressions and pathophysiological mechanisms. The most notable group of CAAs has been termed ‘Anomalous coronary artery originating from the opposite sinus of Valsalva’ (ACAOS), a rare congenital heart disease that is associated with sudden cardiac death and ischemia.&#x0D; We present the case of an 80-year-old man presenting with inferior STEMI having a single coronary ostium and a rare variant of the coronary artery origin belonging to type A4d with an interatrial LAD course according to Angelini's classification. This abnormal finding was managed conservatively and the patient underwent successful drug-eluding stent implantation in the culprit right coronary artery in its middle portion.