Patent ductus arteriosus (PDA) at childhood is one of the five major and frequent congenital abnormalities, but it can be rarely seen in adults. Pulmonary hypertension (PHTN) and other presentations such as heart failure and edema are the identified complications of longstanding PDA, but adult case with no permanent heart symptoms and PHTN was rare. We reported a rare case of with an obvious PDA and normal pulmonary pressure.
A 61-year-old woman presented with dyspnea (New York Heart Association class 2), chest pain, and lower limb edema. Echocardiogram showed; normal left ventricular chamber size and function, normal size of both atria. Furthermore, an obvious PDA (diameter = 6-7 mm) connecting the aortic arch to the pulmonary artery was reported in echocardiography. No lung congestion and evidence for PHTN was reported by computed tomographic angiography [Pulmonary capillary wedge pressure (PCWP) = 30 mmHg]. The patient was treated with antihypertensive drugs and after 1 and 3 months follow-up, edema and other symptoms were resolved.
Finally, we conclude that PDA in adulthood can present with nonspecific cardiovascular symptoms, and it seems that PHTN is not a fixed echocardiographic finding in these patients.
Adults; Edema; Patent Ductus Arteriosus eri; Pulmonary Hypertension
Polyneuropathy, organomegaly, endocrinopathy, monoclonal syndrome (POEMS) is a rare paraneoplastic syndrome associated with plasma cell dyscrasia.
A 48-year-old man presented with a 1-year history of paresthesia and progressive weakness of extremities. Diagnosis of POEMS syndrome was made for him on the basis of clinical presentation, additional physical findings, typical sclerotic bone lesion, and bone marrow findings. In last admission, he explained episodes of dyspnea and chest pain that associated with frequent premature ventricular contraction in his electrocardiograph. Patient heart monitoring showed some episodes of complete heart block. Infra-His atrioventricular block in electro-physiologic study was detected. He had no history of ischemic heart disease. His cardiopulmonary findings on examination were normal. All results of cardiac biomarkers and serum electrolytes and repeated echocardiography were within normal range. Cong red staining of rectal fat pad biopsy was negative. After pacemaker insertion radiation of sclerotic bone, lesion started for him, but radiotherapy was ineffective, and he expired with respiratory failure. Complete heart block in POEMS syndrome has not been reported previously, and it is the first POEMS case with complete heart block.
Complete heart block is a cardiac manifestation of POEMS syndrome.
Complete Heart Block; POEM Syndrome; Multiple Meloma
Interrupted aortic arch (IAA) is defined by a lack of the luminal continuity between the ascending and descending thoracic aorta. It is a rare, severe congenital heart defect which without surgery is associated with high mortality in the neonatal period. The aims of this study were to present a case with IAA who was alive until the age of 59 years without any surgical intervention and to review the literatures that have presented IAA cases.
The patient was admitted with respiratory distress and pulmonary edema. Echocardiography showed the sever stenosis in aortic valve and sever left ventricular dysfunction. Cardiac catheterization and angiography confirmed interrupted aorta (type A). The descending thoracic aorta was supplied by extensive collateral vessels from the vertebrobasilar system down to the posterior chest wall and the spine. Surgical correction including coronary artery bypass graft and aortic valve replacement and repair of interruption of the aorta was performed. Three weeks later the patient was died due to uncontrollable gastrointestinal bleeding and hospital acquired pneumonia. We described diagnosis and management of our case.
This case was very interesting for us, because the patient had not been diagnosed until the recent presentation. Similar cases with this diagnosis do not reach adulthood, but our patient was alive up to 59 years of age.
Interrupted; Aorta; Aortic Valve Stenosis; Thoracic Aorta; Aortic Arch
The severity of ischemia/reperfusion injury determines the neurologic outcome after successful cardiopulmonary resuscitation.
We present a case of prolonged open-chest resuscitation who survived without neurologic sequel. Multiple applied strategies to limit the deleterious effects of ischemia and reperfusion injury, that is, infusion of magnesium sulfate and mannitol, protective lung ventilation and optimal postoperative pain control prevented the end organ damage in this patient. During the 40 min open-chest resuscitation, ventricular defibrillation was successfully attempted with extrathoracic paddles.
The appropriate use of pharmacologic and non-pharmacologic protective strategies could modify the inflammatory cascade and minimize the deleterious effects of reperfusion after prolonged periods of ischemia. The successful defibrillation in this patient warrants the use of standard paddles in open-chest surgeries where surgical small paddles are not available.
Resuscitation; Ischemia; Reperfusion; Neuroprotection; Addiction; Extrathoracic Defibrillation
Tachycardia-induced cardiomyopathy (TIC) is a rare cause of dilated cardiomyopathy (DCMP). The diagnosis can be missed because tachycardia is a common symptom in DCMP.
We reviewed a case 5-year-old with palpitation and dyspnea with symptoms and signs of heart failure that diagnosed as DCMP initially. Then, in the evaluation for cause of tachycardia, atrial tachycardia was detected. Hence, treatment with flecainide was started and after 3 months, left ventricular (LV) systolic function and symptoms of the patient was relieved.
TIC should be suspected in all patients with unexplained LV dysfunctions in the setting of a persistent tachyarrhythmia.
Dilated Cardiomyopathy; Heart Failure; Tachyarrhythmia
Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome (ACS) and sudden death. It usually occurs in young women during the peripartum period; however, it had also been reported in older aged males having risk factors for atherosclerotic coronary artery disease.
This case describes a 69-year-old male patient who presented with manifestations of ACS due to a spontaneous dissection of the Shepherd’s Crook right coronary artery (RCA), which was successfully managed with percutaneous coronary intervention (PCI) and stenting. At the 6th month follow-up, the patient remained chest pain free.
Patients with SCAD in the presence of ongoing ischemia can be treated with PCI and stenting.
Acute Coronary Syndrome; Percutaneous Coronary Intervention; Shepherd’s Crook Right Coronary Artery; Spontaneous Coronary Artery Dissection
Primary pulmonary artery sarcomas are very rare and their histologic type called leiomyosarcoma is even rarer. These tumors are frequently misdiagnosed as pulmonary thromboembolism in clinical settings. Many patients receive anticoagulant therapy without response, and many are diagnosed postmortem only. Most of the tumors reported in the literature have involved the right ventricular outflow tract and the main pulmonary trunk, often extending into the main pulmonary artery (MPA) branches.
A 64-year-old woman presented with weakness, fatigue, malaise, dyspnea, and marked elevation of pulmonary artery pressure was admitted to our hospital. She was initially diagnosed with chronic pulmonary thromboembolism, and chest computed tomography (CT) scan revealed lobulated heterogeneous left hilar mass extended to precarinal and subcarinal space. Magnetic resonance imaging (MRI) demonstrated a polypoid lesion at the trunk with extension to left MPA and its first branch. The patient was operated, and a yellowish-shiny solid mass in pulmonary trunk was seen intra-operation and pulmonary endarterectomy was performed. Her tumor was pathologically diagnosed as pulmonary artery leiomyosarcoma.
Clinicians must consider pulmonary artery sarcoma when making the differential diagnosis for patients with pulmonary artery masses. The clinical prediction scores and the CT and MRI findings can help identifying patients with pulmonary artery sarcoma.
Hypertension; Leiomyosarcoma; Pulmonary Artery; Pulmonary Embolism
Bicuspid aortic valve (BAV) is one of the most common and important congenital heart disorders in adults. If a patient with congenital disorders is not diagnosed early, the patient’s disease may progress to a severe condition and thus diagnosis of the main disorder will be rendered difficult.
A 34 year-old male patient referred to a referral medical care unit for cardiac electrophysiological study with cardiac shock due to complete heart block 3 months ago and he underwent Dual-Chamber permanent pacemaker (PPM) implantation. Thick and calcified bicuspid AV with invasion to interventricular septum, moderate to severe valve insufficiency (AI), severe aortic valve stenosis (AS), and dilated ascending aorta were observed at his echocardiography. Aortic valve replacement (AVR), aneurysm of ascending aorta, root replacement with tube graft (Bentall Procedure), and also a 3 chambers intracardiac defibrillator (ICD) were used. After 2 weeks of operation, he was discharged and at the first post-hospitalization visit (1 week later), his cardiovascular condition was acceptable.
Thick calcified aortic root is a less studied and potential contributing risk factor for AV block after AVR. Therefore, in candidates of aortic valve replacement, considering conductive disorders, especially in patients with calcified valve, is mandatory. Irreversible AV block requiring PPM implantation is a rare condition following AVR.
Atrioventricular Block; Bicuspid Aortic Valve; Calcified Valve
Obesity is a common health problem in both developed and developing countries. There are many unconventional therapies, including herbal medicine, to treat this condition. Some people believe that herbal medicines are safe. This case and review is about adverse complication of treating obesity with some herbal medicine.
A 19 year old male with sever obesity (120 kg) used green tea (15 cups of green tea per day) and an intensive dietary regimen to lose weight. He lost 30 kg after 2 months. At that time, one day after usual exercise he suddenly lost consciousness due to left ventricular fibrillation.
Use of herbal medicine for weight reduction is not always safe. Moreover, for some herbal medicine the risk is sufficient to shift the risk-beneﬁt balance against the use that medicine.
Herbal Medicine; Sudden Death; Complication; Obesity
One of the rare aortic congenital abnormalities is right sided aortic arch which is occurred in approximately 0.1% of the population. The anomalous origin of the subclavian arteries has also been reported.
In this study, a case of a right-sided aortic arch with anomalous left subclavian artery origin from the cerebral arteries is presented which was diagnosed in Computed tomography angiogram (CT angiogram) and angiography of a 10-year-old boy referred due to recurrent chest pains during two weeks before admission and pulselessness of his left upper limb and left carotid artery.
Many of the congenital vascular anomalies may have no obvious signs or symptoms and therefore the exact history and physical examination could help us in better diagnosis. Besides, some of these anomalies are associated with other abnormalities of other body organs particularly the cardiac system.
Congenital Anomaly; Great Arteries; Pediatric
Sub-acute left-sided bacterial endocarditis is a serious condition that may present with variable clinical manifestations. Its symptoms include both sterile and infected emboli, and various immunological phenomena.
This report presents a 55 year old man with frequency and dysuria after a lithotripsy and several admissions with urosepsis. Due to the suspicion of infective endocarditis echocardiography was done which confirmed streptococcus group B endocarditis.
Streptococci group B is one of the rare causes of infective endocarditis, but it was observed after various producers such as lithotripsy.
Group B Streptococci; Infective Endocarditis; Lithotripsy
Coronary artery spasm is a transient narrowing of coronary arteries that slows or stops blood flow through the artery.
We present a 42-year-smoker man without any medical problem who developed syncope. Coronary angiography revealed diffuse significant narrowing of proximal left anterior descending artery (LAD), 90% osteal stenosis of large obtuse marginal (OM), 90% diffuse narrowing of proximal right coronary artery (RCA), which was relieved by intracoronary administration of nitrate. He was discharged on calcium channel blockers and nitrates but one month later developed syncope again and died.
Multivessel coronary artery spasm should be considered in young smoker patients without any other coronary risk factors who present with syncope.
Syncope; Implantable Cardiac Defibrillators; Variant Angina
Truncus arteriosus communis (TAC) is a rare heart disorder with the prevalence of approximately 1%, mostly in male newborns. In this disease, aorta and pulmonary artery have not been separated during fetus development and both originate jointly from left ventricle. In addition, various disorders are reported like ventricular septal defect (VSD), mitral and tricuspid valves defects, aortic septal defect (ASD), reduction of lung and lung vessels’ resistance, pulmonary hypertension, increase in heart rate, high perspiration, bad digestion, and tetralogy of Fallot.
Parents of deceased patient were referred for genetic counseling after the death of third girl due to severe cardiac disorder. Cardiologist declared the disease in deceased girl as TAC based on findings along with VSD, ASD and hypoplastic aortic arch which resulted to death in the first day of birth.
There was no chromosomal disorder in chromosome analysis of patient’ skin. Parents were interested to have another child, so they were referred to university's Genetic Counseling Center to become aware of their next child’s condition. This disorder is genetically heterogeneous and multifactorial and because all external factors are not recognized, the accurate estimation of risk is not possible and the probability of risk for the next child is about 10% to 20%.
Heart Disorder; Truncus Arteriosus Communis; Genetic Counseling
Isolated intrapericardial LAA aneurysm is a rare cardiac anomaly which manifests with angina, dyspnea on exertion (DOE), systemic embolization, arrhythmia, and congestive heart failure.
A 30-year-old female and a 46-year-old male were referred for evaluation of abnormal cardiac contour on chest radiograph and echocardiographic findings and non-specific symptoms. Transesophageal echocardiography suggested left atrial appendage (LAA) mass filled with clots. The mass had no compression on cardiac chambers and global ejection fraction was within normal limits. The intraoperative diagnosis was isolated congenital LAA aneurysm. After confirmation of the diagnosis, it was resected. She was discharged with uneventful postoperative course. At follow-up she was asymptomatic.
These cases demonstrate the role of on-time surgical approaches in the prevention of fatal complication of this rare cardiac anomaly.
Left Atrial Appendage; Aneurysm; Clot
Pneumopericardium is a rare complication following thoracic trauma, and urgent treatment is rendered necessary when it causes tension pneumopericardium due to cardiac tamponade.
The case presented here is a right pericardial laceration with tension pneumopericardium due to falling from a height, presenting to the emergency clinic of our hospital with hemodynamic instability.
Pneumopericardium that might develop due to blunt thoracic trauma can easily be diagnosed, may result in cardiac tamponade, and is a potentially fatal pathology without treatment.
Pneumopericardium; Trauma; Cardiac Tamponade; Fall
Coronary artery anomalies are a rare type of congenital anomalies with an incidence of 1.3% during routine cardiac catheterization. Anomalous origin of the coronary arteries is considered an incidental finding without clinical significance. This case describes a patient in whom evaluation of chest pain revealed an obstructive left anterior descending artery as well as an anomalous right coronary artery arising from the left coronary sinus. The patient underwent successful percutaneous coronary intervention of the left anterior descending artery and was discharged home free of angina 3 days later.
Anomalous Right Coronary Artery; Obstructive Left Anterior Descending Artery; Percutaneous Coronary Intervention
Several studies have emphasized thrombosis associated with thrombocytopenia as a potentially fatal complication of heparin. A number of agents are used for this condition. As a new oral, reversible direct thrombin inhibitor, dabigatran has been approved for short-term thromboprophylaxis after elective hip and knee replacement surgery. We present a case of dabigatran administration in a patient with femoral fracture.
A 67-year-old woman referred to the orthopedic ward of Shariati Hospital (Isfahan, Iran) due to femoral fracture following an accident. Immediately after surgery, she was found to be suffering from deep vein thrombosis (DVT) in her lower extremity despite sufficient prophylaxis by enoxaparin. Laboratory data showed severe thrombocytopenia. Considering the clinical history, an initial diagnosis of heparin-induced thrombocytopenia was made. Doppler ultrasound confirmed the diagnosis. Heparin was thus replaced with dabigatran which increased platelet count to the normal range and improved DVT.
Dabigatran can be a life-saving treatment in heparin-induced thrombocytopenia. However, it is contraindicated in patients with renal dysfunction since it may cause potentially catastrophic results.
Heparin Induced Thrombocytopenia; Heparin; Enoxaparin; Dabigatran
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.
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.
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.
Sinus Valsalva; Aneurysm; Cardiac Tumor
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.
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.
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.
Saccular Aneurysm; Aortic Dissection; Pseudoaneurysm; Aneurysm
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.
Anomalous Origin of Coronary Arteries; Mitral Stenosis.
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.
Jailed wire; Stent; Retrieval
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.
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.
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%.
Holt-Oram Syndrome; Hand- Heart Syndrome; T-BOX.
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.
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).
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.
PTMC; Dextrocardia; Surgical Commissurotomy
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.
Perforation; Dissection; Thrombosis; External iliac artery; Acute arterial occlusion