Tetralogy of Fallot with Cor Triatriatum Dexter in an Adult Patient: A Case Report

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect with only approximately 3% of uncorrected patients surviving past age 40. In this case report, we present a 48‐year‐old mentally retarded man suffering from congenital spastic quadriplegia who was diagnosed with a unique combination of symptomatic TOF and cor triatriatum dexter (CTD). Reduced preload because of CTD with spastic quadriplegia that prevented physical exertion is believed to have facilitated this patient's unusually long survival.     

Diagnosis of a Very Rare Variant of Cor Triatriatum Dexter by Contrast Echocardiography: A Case Report

Cor triatriatum dexter is a rare congenital abnormality in which the right atrium is divided into two chambers by a membrane. A rare variant of cor triatriatum dexter where the membrane is attached to the left of the superior vena cava at one end and to the right of inferior vena cava and coronary sinus at the other end has been described only once before. We describe here a case of this very rare variant of cor triatriatum dexter that was diagnosed using contrast echocardiography.     

Unique Sail‐Like Structure of Cor Triatriatum Dexter in Three‐Dimensional Echocardiogram

Cor triatriatum dexter (CTD) is an extremely rare congenital condition arising from the persistence of the right valve of the sinus venosus. It divides the right atrium (RA) into 2 separate chambers. We report a case of a 50‐year‐old man who had an incidental finding of CTD on transesophageal echocardiogram. An incomplete membrane of the RA was seen, and three‐dimensional echocardiogram delineated the structure clearly as a triangular sail‐like structure with multiple orifices and a fenestration.     

巨大左心房患者的经皮二尖瓣球囊成形术

本文介绍了13例巨大左心房患者的经皮二尖瓣球囊成形术(PBMV).认为手术成功的关键是房间隔穿刺,但已不能采用常规的方法.作者探索了正中法、右侧法和左侧法,并认为右侧法最好.巨大左心房者常为风湿性心脏病二尖瓣严重狭窄的晚期表现,病情重,合并症多,全身情况差,无法耐受开胸手术,而一般内科治疗效果不好.虽然PBMV也有一定风险,但可能是目前唯一较好的治疗方法.本组病例效果令人满意.     

A Combination of Two Simultaneous Tachycardias in the Right Atrium Close to the Atrio-Ventricular Node and Within the Coronary Sinus in a Post-Operative Cor Triatriatum Patient

A 71-year-old male was referred to another hospital for dizziness. A bradycardia -tachycardia syndrome and Cor triatriatum were detected, and an operation to resect the membrane in the left atrium and implant a pacemaker epicardially was performed. However, no suitable site could be found on either atria and therefore, a single chamber ventricular pacemaker was implanted. In the electrophysiological study performed in our hospital, we could not detect any atrial potentials in either atria, excluding the region close to the His bundle (HB) and within coronary sinus (CS), in spite of extensive catheter mapping. A regular atrial rhythm with a cycle length of 820 ms, which was synchronous with the rate of the QRS complex on the surface ECG, was recorded only at the HB. Meanwhile, the CS catheter recording exhibited regular focal activity with a cycle length of 150 ms, and this focal activity did not conduct to the atrium close to the HB. Furthermore, this activity was dissociated from the ventricular activity recorded from the CS catheter. During an isoproterenol infusion, an atrial tachycardia with a cycle length of 380 ms was recorded only at the HB, and the twelve-lead ECG exhibited a regular tachycardia with the same cycle length as this tachycardia. Meanwhile, the focal activity within the CS persisted without any change in the cycle length. These findings suggested that there was dissociation between the right atrium (RA) and CS. Furthermore, partial atrial standstill was observed in both atria, excluding the RA close to the atrio-ventricular (AV) node and area within the CS. These rare electrophysiological features were considered to play an important role in the genesis of a simultaneous combination of the two tachycardias at their respective sites.     

The First Turkish Case of Hypoparathyroidism,Deafness and Renal Dysplasia (HDR) Syndrome

Hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is an autosomal dominant genetic disorder characterized by hypoparathyroidism, sensorineural deafness and renal dysplasia. We herein present the first Turkish patient with HDR syndrome, who has a p.R367X mutation. This report indicates that p.R367X is not a mutation specific for the Far Eastern populations and also that urological findings in infants with hypoparathyroidism should be carefully examined because clinical findings relating to the p.R367X mutation may show a variable age of onset.     

Liver injury due to tetrabamate (Atrium): an analysis of 11 cases

BACKGROUND: Tetrabamate (Atriums), widely used in the treatment of tremor and ethanol-withdrawal symptoms, has been incriminated as a potential cause of reversible acute hepatitis. OBJECTIVE: We report here on 11 patients who experienced tetrabamate-related liver injury, in order to evaluate their clinical, histopathological and evolutive features. PATIENTS AND METHODS: Between 1987 and 1998, 34 cases of tetrabamate-associated acute hepatitis were spontaneously reported to the regional pharmacovigilance center of Lyon. Eleven cases were considered probably to be drug-induced. RESULTS: There were three males and eight females aged 31-82 years (mean, 57 years). The duration of treatment ranged from 33 to 206 days, and indication for treatment was depressive disorders, tremor or prevention of alcohol withdrawal symptoms. Clinical symptoms were asthenia (n = 9), jaundice (n = 3) and/or diffuse rash (n = 3). The pattern of liver injury was cytolytic (n = 10) or cholestatic (n = 1). Three patients presented biological features of hepatic failure. A percutaneous liver biopsy was performed in six patients. Histological examination of the liver specimen showed a large spectrum of lesions: massive hepatocellular necrosis (n = 1), centrilobular and nonconfluent hepatocyte necrosis (n = 2), intracellular cholestasis (n = 3), and granulomatous hepatitis (n = 1). Tetrabamate was discontinued in all patients. In seven patients, a complete recovery was observed 3 weeks to 3 months after drug withdrawal. Two patients, despite a rapid improvement of liver function tests, died from unrelated causes. The remaining two patients died from irreversible hepatic failure. CONCLUSIONS: Our data strongly suggest that tetrabamate may induce acute liver injury, which may eventually result in life-threatening liver failure.     

Case report: First evidence of human zoonotic infection by Onchocerca lupi (Spirurida, Onchocercidae)

In the past decades, cases of canine ocular onchocercosis have been reported worldwide, particularly in the United States and Europe. Onchocerca lupi, originally described from a wolf, has been implicated in some of these cases, and its zoonotic role has been hypothesized on the basis of the reexamination of two cases of human ocular onchocerciasis. In the present study, we describe, for the first time, the occurrence of O. lupi in the subconjunctival region of the human eye in a patient from Turkey. The nematode was identified as O. lupi based on its morphology and molecular phylogenetic analysis of partial cox1 and 12S ribosomal DNA genes. The results suggest that O. lupi should be considered in the differential diagnosis of other eye parasitic infections in humans. The role of dogs as natural hosts of O. lupi and the vectors of this zoonotic parasite need to be investigated.     

Complete Genome and Molecular Characterization of a New Cyprinid Herpesvirus 2 (CyHV-2) SH-01 Strain Isolated from Cultured Crucian Carp

Cyprinid herpesvirus 2 (CyHV-2) is a causative factor of herpesviral hematopoietic necrosis (HVHN) in farmed crucian carp (Carassius carassius) and goldfish (Carassius auratus). In this study, we analyzed the genomic characteristics of a new strain, CyHV-2 SH-01, isolated during outbreaks in crucian carp at a local fish farm near Shanghai, China. CyHV-2 SH-01 exhibited a high sensitivity to goldfish and crucian carp in our previous research. The complete genome of SH-01 is 290,428 bp with 154 potential open reading frames (ORFs) and terminal repeat (TR) regions at both ends. Compared to the sequenced genomes of other CyHVs, Carassius auratus herpesvirus (CaHV) and Anguillid herpesvirus 1 (AngHV-1), several variations were found in SH-01, including nucleotide mutations, deletions, and insertions, as well as gene duplications, rearrangements, and horizontal transfers. Overall, the genome of SH-01 shares 99.60% of its identity with that of ST-J1. Genomic collinearity analysis showed that SH-01 has a high degree of collinearity with another three CyHV-2 isolates, and it is generally closely related to CaHV, CyHV-1, and CyHV-3, although it contains many differences in locally collinear blocks (LCBs). The lowest degree of collinearity was found with AngHV-1, despite some homologous LCBs, indicating that they are evolutionarily the most distantly related. The results provide new clues to better understand the CyHV-2 genome through sequencing and sequence mining.     

Isolated trigeminal neuropathy revealing the presence of Sharp's syndrome or MCTD (mixed connective tissue disease) (author's transl)]

Isolated neuropathies of 5th cranial nerves are rare apart from primary facial neuralgia. A case is reported, in which Raynaud's syndrome was also present, that enabled a diagnosis of mixed connective tissue disease to be established. The authors underline the importance of undertaking complete immunological investigations in order to decide whether there is a particular type of systemic disease present, which could be responsible for these special types of trigeminal nerve disorders.     

Heavy Chain Disease of the {micro} (ggrM) Type: Report of the First Case

The serum of a patient with chroniclymphocytic leukemia, and amyloidosis,was found to contain an unusual proteinwith µ chain antigenic determinants. Itwas devoid of light chains and waspresent in the form of multiple disulfidelinked polymers. The patient also had akappa Bence Jones protein in the serumand urine. The possibility is raised thatthe heavy chain lacks a portion of thechain necessary for coupling with lightchains.

Submitted on March 11, 1970 Accepted on March 20, 1790     

Percutaneous implantation of permanent pacemakers (author's transl)

2 methods for the implantation of permanent pacemakers with percutaneous puncture of the subclavian vein are described. The first one was used in 44 patients with the aid of an introducing catheter-set Desilets-Hoffman consisting of guide-wire, inner Teflon dummy catheter and outer thin-walled sheath; through this a shoulder-less electrode catheter no. 8 could be inserted for permanent pacing. The other technique was applied to 20 patients and used thinner special electrodes no. 6F, which could be introduced directly through a plastic cannula inserted with a puncture-needle. The advantages are: diminished risk of infections, local anesthesia instead of general anesthesia, applicability by the cardiologist in the catheterization-laboratory or under a simple fluoroscopy-unit, short stay of patients in the hospital without transfers to other departments, few personnel (1 scrubbed doctor, 1 non-scrubbed nurse), recognition of venous anomalies (singular left superior caval vein) without useless incisions for the patient. Complications such as pneumothorax, puncture of the subclavian artery, and dislocations of the electrodes have also been encountered.