Cor triatriatum sinister with left superior vena cava, unroofed coronary syndrome and valvular pulmonary stenosis / Cor triatriatum sinistrum con vena cava superior izquierda, síndrome coronario destechado y estenosis de la válvula pulmonar

Abstract A 25-year-old woman with a history of recent dyspnea and palpitation was admitted to our center. Transthoracic echocardiography (TTE) showed an echogenic and septal muscular ridge along the left atrium which were indicative of cor triatriatum sinister (CTS). Further evaluation with transesophageal echocardiography (TEE) showed that a lower chamber of divided left atrium receiving lower right inferior pulmonary vein, mitral valve, left superior vena cava (LSVC) and unroofed coronary sinus (CS). Also, the lower chamber had an unrestrictive communication with the right atrium. The upper accessory chamber receiving one left and one right upper pulmonary vein and communicated with the right atrium by a small atrial septal defect (ASD). However, upper and lower pulmonary venous systems separated directly from each other by a muscular ridge without the presence of any window or hole to allows blood flow between these two accessory chambers. Although the absence of septum associated with the presence of LSVC and unroofed CS makes our case a unique or very rare type of this complex anomaly in an adult case. In our case, surgical removal of dividing muscular membrane with redirection of LSVC and unroofed CS to the right atrium are warranted.

Resumen Se trata de una paciente de sexo femenino, de 25 años de edad, con antecedentes recientes de disnea y palpitaciones que ingresó a nuestro centro. Un ecocardiograma transtorácico (ETT) evidenció un reborde ecogénico muscular del tabique de la aurícula izquierda indicativo de cor triatriatum sinistrum (CTS). En un estudio más a fondo con ecocardiografía transesofágica (ETE) se evidenció que una cámara inferior de la aurícula izquierda dividida recibía la vena pulmonar inferior derecha, la válvula mitral, la vena cava superior izquierda (VCSI) y el seno coronario (SC) destechado. Además, la cámara inferior tenía comunicación libre con la aurícula derecha. La cámara superior secundaria recibía una vena pulmonar superior izquierda y una derecha y se comunicaba con la aurícula derecha a través de una comunicación interauricular (CIA) pequeña. Sin embargo, los sistemas venosos pulmonares superiores e inferiores se encontraban separados entre sí por un reborde muscular sin la presencia de alguna ventana u orificio que permitiera el flujo de sangre entre estas dos cámaras secundarias. La ausencia del tabique junto con la presencia de la VCSI y el SC destechado hacen que nuestro caso sea un tipo único o extremadamente inusual de esta anomalía compleja en un caso en un adulto. En este caso, se justifica la extirpación quirúrgica de la membrana muscular divisoria con redireccionamiento de la VCSI y el SC destechado a la aurícula derecha.

A rare case of Watson syndrome.

Watson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown color skin lesions that are known as cafe-au-lait spots. We present a rare new variant of the syndrome in an adult patient with severe pulmonary valve stenosis, main, left, and right pulmonary artery aneurysm, short stature, mental retardation, coronary artery disease, and skin lesions. The patient underwent open cardiac surgery with pulmonary valvotomy and aneurysmorrhaphy of the main pulmonary artery and its right and left branches. The postoperative course was uneventful and the six-month follow-up with transthoracic echocardiography revealed no recurrence of aneurysm of repairing pulmonary arteries and good clinical outcome of the patient. Our patient had a unique characteristic of aneurysm of the main pulmonary artery and its both branches that has rarely been reported previously in the medical literature.

Combination of cardiac and carotid glomus tumour: a rare case report.

The most common tumour of the heart is myxoma but paraganglioma (also called glomus tumour in extracardiac sites) in the cardiac position is the rarest of them. While this tumour accounts for 0.8% of all primary benign tumours, the combination of both neoplasms is an exceedingly rare occurrence. Herein, we present a case of combined carotid glomus tumour and left atrial paraganglioma tumour in which respiratory distress was the presenting symptom of cardiac type but carotid tumour was asymptomatic. The case underwent a two-step resection of the neck and cardiac mass with an uncomplicated postoperative course and in the 1year follow-up, no recurrence of tumour in both sites was found on physical exam and imaging studies.

A Rare Location of Pseudoaneurysm in Subaortic Ring after Bentall Operation.

Bentall operation is considered a gold standard of surgery in the treatment of ascending aortic aneurysm. This operation with en-bloc resection of ascending aorta and aortic valve requires aortic valve with two coronary bottoms re-replacements in a prepared composite graft. The four important locations for the pseudoaneurysm include proximal and distal composite graft anastomosis and two coronary bottom sites. However, many complications have been reported with this technique but the most serious complication associated with this operation is defined as pseudoaneurysm. We report an exceedingly rare case of subaortic ring pseudoaneurysm in retro composite graft position enclosed by infected surgical that was used to control bleeding in this location. Dehiscence occurred between the aortic ring and the underlying left ventricular muscle. The aortic ring was separated from the underlying muscle by the high tensile strength of sewing ring sutures. The pseudoaneurysm compressing the left atrium without communicating with any cardiac chamber and presenting with high fever (39°C), chills, a few months after Bentall operation. The patient underwent redo operation and repair of the dehiscence's site. The 6-month follow-up revealed no recurrence of a pseudoaneurysm. The uniqueness of this case report is related to the site of pseudoaneurysm between the aortic ring and underlying left ventricular muscle that have not been reported in the medical literature so far.

Association of cardiac enzymes with morbidity and mortality of patients undergoing coronary endarterectomy surgery.

BACKGROUND: The relationship between cardiac enzyme release following coronary endarterectomy (CE) and morbidity and mortality is unclear. Therefore, the present study aimed to investigate the association of cardiac enzymes with morbidity and mortality of patients undergoing CE surgery. METHODS: This was a single-center retrospective cohort study of 475 patients who had undergone off-pump coronary artery bypass graft (OPCABG). The patients were followed up for a mean of 72.99 ± 14.60 months. RESULTS: Among 475 patients undergoing OPCABG, 39 (8.2%) were non-survivors. Non-survivors were younger and had a fewer ejection fraction (EF). Comorbidities were similar in survivors and non-survivors. The crude Cox regression analysis showed that creatine kinase-myocardial band (CK-MB) had a protective effect against mortality, but when adjusted with age, sex, diabetes mellitus (DM), hypertension (HTN), hyperlipidemia, smoking, family history, body mass index (BMI), left main disease (LMD), and EF, this effect disappeared. Troponin in crude and adjusted analysis did not have any significant effect. CONCLUSION: There is no association between CK-MB and troponin and mortality in patients undergoing coronary artery bypass graft (CABG).

Anabolic Steroid Use and Aortic Dissection in Athletes: A Case Series.

The use of anabolic steroids in supraphysiologic doses has grown in the last decade as doping drugs in athletes. The high dose of anabolic-androgenic steroids (AAS) causes cardiomyopathy, hypertension, thrombosis, myocardial infarction (MI), weakness of connective tissue, and its sequelae such as tendon injury and aortic dissection. Dissection of the ascending aorta is an uncommon injury that has been recognized with increasing frequency in bodybuilders in recent years. It has been proposed that such cases commonly accompany the weakening of connective tissue and must be actively evaluated in the presence of anabolic steroid usage. We present a case series of isolated ascending aorta dissection in athletes who were bodybuilders. All cases were evaluated by transthoracic echocardiography (TTE) and laboratory exams. These cases also served as a reminder of the risks of ascending aorta dissection with AAS, especially in strength athletes who place high demands on their musculoskeletal structures. The results of the current study suggested that anabolic steroid abuse may be associated with detrimental effects on the myocardium represented as cardiomyopathy or atherosclerotic changes in the coronary artery as MI. These findings also strongly suggest that anabolic steroid treatment predisposes the individual to aortic dissection, especially when the patients are exercised.

Traumatic Hemolytic Anemia after Valve Surgery: a Case Report.

Hemolytic anemia is an uncommon complication after mitral valve repair. We present a case of a 55-year-old man who presented with post-operative hemolytic anemia after mitral valve repair with prosthetic ring. The hemolytic anemia improved after the patient had the prosthetic ring removed and the valve replaced by a prosthetic mitral valve. However, the post-operative course of the redo operation was complicated by acute renal failure and respiratory dysfunction, but the hemolytic anemia was finally abolished and the patient was discharged 20 days post-operatively in good condition.

Right Atrial Blood Cyst with Stones Suspended from the Coronary Sinus.

Cardiac blood cysts are rare benign neoplasms, usually involving the cardiac valves and are remnants of the Chiari network. They are usually detected in the first six months of life and rarely occur in children or adults. We report a 76-year-old male patient who was referred to the Imam Ali Hospital affiliated with Kermanshah University of Medical Sciences, Kermanshah, Iran, in 2018 with dyspnoea. Transthoracic echocardiography revealed a small patent foramen ovale (PFO) and a circumferential mobile cystic mass in the right atrium, with the impression of a tumour or thrombus. The patient underwent open-heart surgery with cardiopulmonary bypass to repair to PFO and remove the intra-atrial lesion. During surgical examination of the right atrial cavity, a blood cyst containing small stone-like structures on the coronary sinus valve of the right atrium was found. The post-operative course was uneventful and no recurrence of tumour was detected during six months of follow-up. To the best of our knowledge, this is the first reported case of a right atrial blood cyst with a few nodule-like stones in an adult with PFO in Iran and the second case in an adult with PFO worldwide.