BACKGROUND

Sinus of Valsalva aneurysm (SVA) may be congenital or acquired. They could mimic ventricular tumor symptoms and cause signs and symptoms of ventricular outflow tract obstruction. They may also involve the conduction system and cause palpitations or syncopal episodes. Both transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) serve as quick, noninvasive methods to provide information on size and location of aneurysmal dilatation and cardiac chamber involvement. These methods can identify any associated anomalies or complications. This study presents a patient with unruptured SVA.

CASE REPORT

A 46-year-old man, who had been suffering from nonspecific symptoms such as exercise intolerance and weakness for a few months, referred to our clinic in Isfahan (Iran). In TTE, a large mass was observed in the right ventricle. SVA was suspected after meticulous probing. This diagnosis was confirmed by TEE and computed tomography angiography. At open heart surgery, an SVA with a lot of clots it was removed.

CONCLUSION

SVA must be kept in mind when a tumor-like mass is observed in the right ventricle. Detailed evaluation would thus be necessary to rule out SVA and to prevent wrong diagnosis and treatment that can sometimes be catastrophic.

BACKGROUND

In true aneurysm, the wall of aneurysm is composed of the normal histological components of aorta. A false aneurysm (pseudoaneurysm) represents a rupture which does not contain the normal histological components of aorta. It is a fibrous peel that has formed from a small perforation of aorta. We describe an unusual presentation that has signs which some of them are only manifested in true aneurysm and some others only in pseudoaneurysm.

CASE REPORT

An 85-year-old man underwent elective coronary angiography for chest pain work-up. Our evaluation by invasive angiography and CT angiography showed aortic dissection. In surgery we found that dissection flap was composed of some parts of intima and media layers. These signs leaded to confusing symptoms. Localized bulging of ascending aorta had continued to brachiocephalic artery (transverse arch involvement). Dissection flap was composed of some part of intima and media layers. It was a strange case, it was not solely a perivascular hematoma and it did not have all three layers of aorta wall. Partial aorta replacement was performed. The operation and recovery was uneventful.

CONCLUSION

This unusual presentation of disease has not been mentioned in literatures. Our experience can help to manage similar cases. This case was the first unusual presentation of its type.

Congenital coronary anomalies are presented in approximately1% of patient referred for cardiac catheterization. Among the congenital coronary anomalies, a separated anomalous origin of all the coronary arteries from the right sinus of valsalva is very uncommon. We report a rare occurance of simultaneous occurence of mitral stenosis with ectopic origin of left main stem coronary artery from right sinus of Valsalva.

Side branch wiring is not infrequently used to protect side branch flow after main vessel stenting. Rarely, it becomes difficult to retrieve the jailed wire behind the stent and therefore it may even be detached and remained in the circulation. This article presents a report of such a case and reviews treatment options.

BACKGROUND

The Holt-Oram Syndrome (HOS) or the Heart-Hand syndrome is considered as an overt autosomal hereditary disease with a complete influential effect and variable expression that emerges due to a genetic mutation. It can be vividly characterized by heart disorders and deficiencies in hand structure. Despite the existing genetic heterogeneity, the variable integration between HOS and the T-BX5 gene from the T-BOX Gene Complex has been observed during which various mutations have been reported in the affected patients. The T-BOX Gene Complex is located on chromosome 12 (12 q 24.1), and is able to encode a copying factor. That has a conservative motive with 180 amino acids. The deficiencies in only 1/3 of patients have been observed caused by the mutation of this gene.

CASE REPORT

This case was a 10-year-old child with hand disorders, incomplete growth of clavicle, moving problems in elbow joint and shoulder, disorder in ventricle and auricle walls. The disease was diagnosed as HOS, based on clinical examination and drawing the family tree diagram. It was impossible to investigate molecular mutation due to inaccessibility to the patient.

CONCLUSION

By investigating the family members and their deficiencies and imaging disorder variables according to different reports as well as parents’ state of health, the HOS can be defined as an overt heredity resulting from a new mutation in the germinating layer of sexual cells in one of the parents. There is a weak possibility that the disease results from a mosaic mutation in the male parent's testicle or the female parent's ovum. In this case, the probability for the disease to be repeated in parents’ next children will be guessed between 1 and 50%.

BACKGROUND

Dextrocardia situs inversus refers to the heart being a mirror image situated on the right side of the body. Distorted cardiac anatomy provides technical difficulties during fluoroscopy-guided transcatheter procedures. This is even more difficult in the case with percutaneous transvenous mitral commissurotomy (PTMC). Mitral valvuloplasty is a minimally invasive therapeutic procedure to correct an uncomplicated mitral stenosis by dilating the valve using a balloon. Here, we describe a case of a 25 years-old male with situs inversus and dextrocardia.

CASE REPORT

A 25 years-old man, having situs inversus and suffering from mitral stenosis was referred to hospital for PTMC. His initial examination findings were unremarkable and an electrocardiographic (ECG), trans-esophageal and transthoracic echocardiographic evaluation were performed. Mitral valve (MV) was dome shape and severely stenotic with mild mitral regurgitation (MR). Left ventricularejection Fraction (LVEF) was about 40%, Femoral arterial and venous punctures were made on the left side; the left femoral artery and vein were cannulated with a 5F arterial and 6F venous sheaths, respectively. Then special maneuvers were done to solve the mitral valve stenosis. At the end of the procedure, no MR was documented by checking LV angiogram and there were no signs of mitral stenosis (MS).

CONCLUSION

Mirror-image dextrocardia, as in our case, has been estimated to occur with a prevalence of 1:10,000. However, there are only a few case reports in the literature on PTMC in similar settings. This might be due to the fact that many of these patients undergo surgical commissurotomy due to the technical difficulties involved in a percutaneous procedure in general. Trans-septal catheterization is considered a technical challenge in anatomically malpositioned hearts, as it is fraught with a higher risk of cardiac perforation. Despite the challenging anatomy, PTMC has been demonstrated to be a safe and feasible option for MS in patients with unusual cardiac anatomy.

Lower extremity complications are the most common problems encountered during transfemoral diagnostic coronary artery angiography. Dissection, thrombosis and perforation of arteries of lower extremities although not uncommon, very rarely occur simultaneously. We did not find any report in the literature in this issue. In this report we describe a case of simultaneous occurrence of all three complications during coronary angiography in one patient. It also represents some of our uncertainties regarding the best management of the patient. In this patient, arterial perforation and dissection was managed conservatively, but we applied an invasive treatment (surgical thrombectomy) for arterial thrombosis with excellent short and long-term results.