Challenging surgery of giant right coronary aneurysm associated with coronary sinus fistula.

Coronary artery fistula is a rare congenital or acquired anomaly. It involves an abnormal connection between the coronary artery and the cardiac chambers or the large thoracic vessels. In some cases, the feeding coronary artery can become extremely dilated. The treatment includes a transcatheter or a surgical intervention depending on the complexity of the anomaly. We present the surgical treatment of the coronary artery to coronary sinus fistula, which includes the complete exclusion of the giant right coronary artery and followed by triple bypass surgery.

An (In)Significant Ventricular Septal Defect and/or Double-Chambered Right Ventricle: Are There Any Differences in Diagnosis and Prognosis in Adult Patients.

A double-chambered right ventricle (DCRV) is an uncommon congenital anomaly: the right ventricle (RV) is divided into two chambers due to the presence of an abnormally located muscular band or anomalous muscle hypertrophy in the subinfundibular part of RV outflow tract, with a variable degree of obstruction. Generally, DCRV is well recognized in childhood and misdiagnosed in adult patients. Transthoracic and/or transesophageal echocardiography are the mx0435;thods of choice for the diagnosis of DCRV. Due to limitations of echocardiography in adult patients, this entity may be missed, particularly if it presents concomitant with other congenital defects, and therefore additional imaging methods such as MRI or cardiac catheterization are required for a definitive diagnosis.

European Society of Cardiology Working Group on Adult Congenital Heart Disease and Study Group for Adult Congenital Heart Care in Central and South Eastern European Countries consensus paper: current status, provision gaps and investment required.

AIMS: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. METHODS AND RESULTS: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005). CONCLUSION: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent.

Women leaders in Cardiology. Contemporary profile of the WHO European region.

Aims: Women's participation is steadily growing in medical schools, but they are still not sufficiently represented in cardiology, particularly in cardiology leadership positions. We present the contemporary distribution of women leaders in cardiology departments in the World Health Organization European region. Methods and results: Between August and December 2020, we applied purposive sampling to collect data and analyse gender distribution of heads of cardiology department in university/third level hospitals in 23 countries: Austria, Azerbaijan, Belgium, Bosnia-Herzegovina, Croatia, France, Germany, Greece, Italy, North Macedonia, Morocco, Poland, Portugal, Russia, Serbia, Slovakia, Slovenia, Spain, Switzerland, Tunisia, Turkey, Ukraine, and the UK. Age, cardiology subspecialty, and number of scientific publications were recorded for a subgroup of cardiology leaders for whom data were available. A total of 849 cardiology departments were analysed. Women leaders were only 30% (254/849) and were younger than their men counterpart (♀ 52.2 ± 7.7 years old vs. ♂ 58.1 ± 7.6 years old, P = 0.00001). Most women leaders were non-interventional experts (♀ 82% vs. ♂ 46%, P < 0.00001) and had significantly fewer scientific publications than men {♀ 16 [interquartile range (IQR) 2-41] publications vs. ♂ 44 (IQR 9-175) publications, P < 0.00001}. Conclusion: Across the World Health Organization European region, there is a significant gender disparity in cardiology leadership positions. Fostering a diverse and inclusive workplace is a priority to achieve the full potential and leverage the full talents of both women and men.

Double orifice mitral valve--A case report.

INTRODUCTION: Double orifice mitrol valve (DOMV) is a very rare congenital heart defect. CASE REPORT: We reported 20-year-old male referred to our center due to evaluation of his cardiologic status. He was operated on shortly after birth for a tracheoesophageal fistula. Accidentally, echocardiography examination at the age of 4 years revealed double orifice mitral valve (DOMV) without the presence of mitral regurgitation, as well as mitral stenosis, with normal dimensions of all cardiac chambers. The patient was asymptomatic, even more he was a kick boxer. His physical finding was normal. Electrocardiography showed regular sinus rhythm, incomplete right bundle branch block. Transthoracic echocardiography (ITE) examination revealed the normal size of the left atrial, mitral leaflets were slightly more redundant. The left and right heart chambers, aorta, tricuspid valve and pulmonary artery valve were normal. During TTE examination on a short axis view two asymmetric mitral orifices were seen as a double mitral orifice through which we registered normal flow, without regurgitation and mitral stenosis. Transesophageal echocardiography (TEE) examination from the transgastric view at the level of mitral valve, showed 2 single asymmetric mitral orifices separated by fibrous tissue, mitral leaflet with a separate insertion of hordes for each orifice. CONCLUSION: The presented patient with DOMV is the only one recognized in our country. The case is interesting because during 16-year a follow-up period there were no functional changes despite the fact that he performed very demanded sport activities. This is very important because there is no information in the literature about that.

Challenges in treatment of postinfarction ventricular septal defect and heart failure.

INTRODUCTION: Acquired ventricular septal defect (VSD) is uncommon, but serious mechanical complication of acute myocardial infarction with poor outcome and high mortality rate in surgically or medically treated patients. CASE REPORT: We report a 58-year-old male patient admitted to our hospital six days following acute inferior myocardial infarction complicated by ventricular septal rupture with signs of heart failure. Coronary angiography revealed 3-vessel disease, with proximally occluded dominant right coronary artery. Transthoracic echo exam revealed aneurysm of a very thin inferior septum and the basal portion of the inferior left ventricular wall, with septal wall rupture. One of the VSD dimensions was 15 mm and left- to right shunt was calculated 2:1. Since the patient was at too high risk for surgical closure, transcatheter closure of VSD was chosen as a better option. Under short intravenous sedation, 24 mm Am- platzer device was implanted percutaneously with transesophageal echo guidance. The post-procedural result revealed a small residual shunt, but it was followed by significant improvement of the patient's clinical status. A 24h Holter ECG monitoring did not show cardiac rhythm or conduction disturbances. Coronary angiography was repeated ten days following the procedure, after hemodynamic stabilization of the patient, with direct stenting of the circumflex artery and the intermediate artery. Ostial left descending artery lesion was left for further functional significance assessment. CONCLUSION: Percutaneous closure with a septal occluder device can be definitive primary treatment for anatomically suitable patients or it can serve as a bridge to surgical treatment.

Cor triatriatum dexter and atrial septal defect in a 43-year-old woman.

Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful. In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.

Exuberant accessory mitral valve tissue with possible true parachute mitral valve: a case report.

INTRODUCTION: A parachute mitral valve is defined as a unifocal attachment of mitral valve chordae tendineae independent of the number of papillary muscles. Data from the literature suggests that the valve can be distinguished on the basis of morphological features as either a parachute-like asymmetrical mitral valve or a true parachute mitral valve. A parachute-like asymmetrical mitral valve has two papillary muscles; one is elongated and located higher in the left ventricle. A true parachute mitral valve has a single papillary muscle that receives all chordae, as was present in our patient. Patients with parachute mitral valves during childhood have multilevel left-side heart obstructions, with poor outcomes without operative treatment. The finding of a parachute mitral valve in an adult patient is extremely rare, especially as an isolated lesion. In adults, the unifocal attachment of the chordae results in a slightly restricted valve opening and, more frequently, valvular regurgitation. CASE PRESENTATION: A 40-year-old Caucasian female patient was admitted to a primary care physician due to her recent symptoms of heart palpitation and chest discomfort on effort. Transthoracic echocardiography showed chordae tendineae which were elongated and formed an unusual net shape penetrating into left ventricle cavity. The parasternal short axis view of her left ventricle showed a single papillary muscle positioned on one side in the posteromedial commissure receiving all chordae. Her mitral valve orifice was slightly eccentric and the chordae were converting into a single papillary muscle. Mitral regurgitation was present and it was graded as moderate to severe. Her left atrium was enlarged. There were no signs of mitral stenosis or a subvalvular ring. She did not have a bicuspid aortic valve or coarctation of the ascending aorta. The dimensions and systolic function of her left ventricle were normal. Our patient had a normal body habitus, without signs of heart failure. Her functional status was graded as class I according to the New York Heart Association grading. CONCLUSIONS: A recently published review found that, in the last several decades, there have been only nine adult patients with parachute mitral valve disease reported, of which five had the same morphological characteristics as our patient. This case presentation should encourage doctors, especially those involved in echocardiography, to contribute their own experience, knowledge and research in parachute mitral valve disease to enrich statistical and epidemiologic databases and aid clinicians in getting acquainted with this rare disease.

Left ventricular noncompaction: clinical-echocardiographic study.

BACKGROUND/AIM: Left ventricular noncompaction (LVNC) is a disorder in endomyocardial morphogenesis, seen either isolated (in the absence of other cardiac anomalies) or in association with congenital heart disease and some neuromuscular diseases. Intrauterine arrest of the compaction of myocardial fibers is postulated to be the reason of LVNC. Recognition of this condition is extremely important due to its high mortality and morbidity that lead to progressive heart failure, ventricular arrhythmias and thromboembolic events. The aim of this study was to determine the prevalence and clinical presentation of LVNC among consecutive outpatients according to clinical and echocardiographyic findings. METHOD: A total of 3,854 consecutive patients examined at the Institute for Cardiovascular Diseases within a period January 2006 - January 2007 were included in the study. All the patients underwent echocardiographic examination using the same equipment (Vivid 7, GE Medical System). Echocardiographic parameters and clinical presentation in patients with echocardiographic criteria for LVNC were analyzed. RESULTS: Analyzing 3,854 consecutive outpatients, using two-dimensional Color Doppler echocardiography from January 2006 to January 2007, 12 patients met the criteria for LVNC. Seven of them were male. The mean age at diagnosis was 45 +/- 15 years. Analyzing clinical manifestation of LVNC it was found that seven patients had signs of heart failure, six had arrhythmias with no embolic events. CONCLUSION: Our results suggest that the real prevalence of LVNC may be higher than expected. New studies have to be done to solve this problem.

A misinterpretation of the left ventricular non-compaction-adult patient with primary pulmonary hypertension.

Non-compaction of the left ventricle is a rare cardiac malformation, defined as a primary cardiomyopathy caused by genetic malformations. Although the pathogenesis of this cardiomyopathy is unknown, there are two possible hypotheses (congenital and acquired) which lead to arrest in intrauterine endomyocardial morphogenesis. We are presenting a case of a 60-year-old woman, with a history of bradyarrhythmia, syncope and cyanosis. Two-dimensional echocardiography showed the thickened myocardium with prominent trabeculations and deep intertrabecular recesses in the two thirds of the apical part of left ventricle walls. The right side cavity was enlarged with hypertrophied wall. Tricuspid regurgitation was moderate. Systolic pressure in the right ventricle was 70mmHg. Catheterization of the right heart showed high pressure in the pulmonary artery. According to publications, this is a very rare case with the presence of possible primary pulmonary hypertension and non-compaction of the left ventricle.

Transecting the aorta to close the aortic prosthetic valve paravalvular space.

The residual aortic prosthetic valve paravalvular space beneath the left coronary artery is very difficult and risky to repair. To improve visualization of this area, we transected the ascending aorta and mobilized both the aortic root and the left coronary artery from the surrounding tissue. Subsequently, we retracted the left coronary artery aside and pulled the posterior aortic root upwards. This maneuver has provided enough space for the safe placement of stitches and the closure of the paraprosthetic defect in this very delicate area.

[Posterior cusp enlargement in mitral valve reconstructive surgery for restrictive valve insufficiency--case report].

INTRODUCTION: According to Carpentier classification, group III of mitral insufficiency is caused by the restrictive motion of the mitral valve. A rheumatic process and a coronary disease are the main causes. It is very important to examine the valve precisely, to define deformities and aetiology in order to make such a valve functional. In rheumatic disease, fibrosis and degeneration deform the entire mitral apparatus. A surgical principle is to re-establish mobility and pliability of the apparatus taking care of the functional anatomy at the same time. Cusp enlargement, chordal mobilization and ring remodelling arejust some of the numerous surgical techniques that could be performed in these patients. CASE OUTLINE: A 55-year-old female patient was admitted to hospital with severe mitral regurgitation. She belonged to NYHA, functional stage 2. Echocardiography revealed that both cusps, mostly posterior, were of reduced size and mobility, resulting in grade 3 regurgitation. We performed posterior cusp enlargement by using autologous pericardium treated in 0.6% of glutaraldehyde solution. Annulus remodelling was done using size 27 flexible Duran ring. Control echocardiography found trivial regurgitation. The patient was discharged on 12th postoperative day in sinus rhythm. CONCLUSION: Surgical reconstruction of the rheumatic mitral valve back to the functional state is a demanding procedure, which, however, provides certain benefit for the successfully treated patient.