Ventricular septal defect and Swyer-James (Macleod's) syndrome together: a case report

Swyer-James (MacLeod's) syndrome is a rare entity that is characterized by the hyperlucent appearance of one or more lobes of one lung, diminished ipsilateral hilar shadow and hardly visible arterial structure on chest radiography. Ventricular septal defect is the most common childhood congenital heart defect and, if left untreated, this will result to irreversible pulmonary hypertension, reversing of the shunt and Eisenmenger's syndrome. In this case report, we describe a 19-year-old female patient who had diagnosis of VSD and Swyer-James syndrome at the same time.     

A case of Swyer-James syndrome with bilateral lesions]

A 16-year-old female was admitted for the treatment of bronchial asthma. She had a history of measles pneumonia at four years of age. Her chest radiograph revealed right unilateral hyperlucency. She was diagnosed as having Swyer-James syndrome from the results of computed tomography, pulmonary angiography, bronchoscopy, and perfusion-ventilation scintigrams, which revealed unsuspected bilateral involvement, and irregularity of severity and distribution. In addition, she had a hypoplastic right upper bronchus, pulmonary hypertension, pneumothorax, and bronchial asthma. The findings of bilateral involvement, irregular distribution, and bronchial lesions strongly suggest that the etiology in this case was damage to the airways caused by measles infection in childhood.     

Pathological impact of hyperpolarization-activated chloride current peculiar to rat pulmonary vein cardiomyocytes

Pulmonary veins (PVs) are believed to be a crucial origin of atrial fibrillation. We recently reported that rat PV cardiomyocytes exhibit arrhythmogenic automaticity in response to norepinephrine. Herein, we further characterized the electrophysiological properties underlying the potential arrhythmogenicity of PV cardiomyocytes. Patch clamping studies revealed a time dependent hyperpolarization-activated inward current in rat PV cardiomyocytes, but not in left atrial (LA) myocytes. The current was Cs⁺ resistant, and was not affected by removal of external Na⁺ or K⁺. The current was inhibited with Cd²⁺, and the reversal potential was sensitive to changes in [Cl⁻] on either side of the membrane in a manner consistent with a Cl⁻ selective channel. Cl⁻ channel blockers attenuated the current, and slowed or completely inhibited the norepinephrine-induced automaticity. The biophysical properties of the hyperpolarization-activated Cl⁻ current in rat PVs were different from those of ClC-2 currents previously reported: (i) the voltage-dependent activation of the Cl⁻ current in rat PVs was shifted to negative potentials as [Cl⁻]_i increased, (ii) the Cl⁻ current was enhanced by extracellular acidification, and (iii) extracellular hyper-osmotic stress increased the current, whereas hypo-osmotic cell swelling suppressed the current. qPCR analysis revealed negligible ClC-2 mRNA expression in the rat PV. These findings suggest that rat PV cardiomyocytes possess a peculiar voltage-dependent Cl⁻ channel, and that the channel may play a functional role in norepinephrine-induced automaticity.     

Persistent iatrogenic atrial septal defect after pulmonary vein isolation

INTRODUCTION: Pulmonary vein isolation (PVI) is widely practiced for treating atrial fibrillation. Transseptal access is necessary with one or even more transseptal sheaths to perform PVI. METHODS: In this prospective study, 31 patients were examined with transesophageal echocardiography before, immediately after and in a 3-, 6- and 12-month follow-up period for evaluation of iatrogenic atrial septal defect (iASD). All patients underwent PVI with double transseptal puncture. RESULTS: An iASD was detected in 27/31 patients (87%) with a maximum diameter of 1.0 mm. After 3 months, the iASDs were completely closed in 26/27 (96.3%) patients. In 1/27 (3.7%) patients, there was an iASD detectable even after 12 months. Clinically no patient suffered from cerebral or cardiac embolism in the follow-up period. CONCLUSIONS: We were only able to find small iASDs with left to right shunting after PVI but none with right to left shunting. iASDs following PVI show a high spontaneous closure rate already at 3 months of follow-up and are not associated with an increased rate of paradoxical embolism.     

A case of Swyer-James syndrome with interesting chest radiographs]

A 28-year-old man was admitted for further examination after overinflation of the right lung was observed on a screening chest radiograph. The chest radiograph on admission showed increased radiolucency of the left lung and overinflation of the right lung, associated with a shift of the mediastinum toward the midline on expiration. High-resolution CT on expiration showed air trapping in the left lung and in part of the right lower lobe. Pulmonary perfusion scintigraphy showed a markedly decreased perfusion in these areas. Pulmonary angiography revealed small left pulmonary arteries with diminished peripheral vascular branches. Bronchoscopy showed no abnormality of either bronchus. Swyer-James syndrome was diagnosed on the basis of these findings. Although bronchial damage due to the lower respiratory tract infection during childhood is considered a very important factor in the pathogenesis of this syndrome, many patients have no history of airway infection, as in this case. This is a rare case of Swyer-James syndrome presenting with marked overinflation of the unaffected lung in an asymptomatic adult. Progression of this compensative overinflation probably involved asymptomatic damage caused by the weakness of the affected lung.     

A case of anomalous course of the pulmonary vein with a tracheobronchus]

A 28-year-old man was admitted to our hospital for further evaluation of a loop-like abnormal shadow in the middle lower lung field on chest X-ray film. Pulmonary angiograms demonstrated staining of the abnormal shadow during the venous phase. The abnormal vein descended from the right upper lobe to the middle lobe and then tortuously made a loop upward to drain into the left atrium. A part of the right apical vein was stenosed. The right apical segmental bronchus directly branched from the trachea. These observations indicate that the abnormalities may have originated in the prenatal period. Cases of anomalous course of the pulmonary vein are very rare.     

A familial variant of the Scimitar syndrome with a meandering pulmonary vein

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.     

A case of hypereosinophilic syndrome associated with pulmonary infarction and hepatic vein obstruction (Budd-Chiari syndrome)

A 21-year-old man was admitted in March 1987 with low grade fever and chest pain. Eosinophilia had been pointed out and PIE syndrome was diagnosed in another hospital a month before admission. Steroid therapy had been started. On the first admission, the chest roentgenogram showed bilateral pleural effusion and a nodular shadow in the left lower lung field. Open lung biopsy was performed and a diagnosis of pulmonary infarction was made. Eosinophilia, low grade fever and chest pain were improved by steroid therapy. He was discharged in April 1987. He was readmitted in September 1987 because of fever, back pain and abdominal distension. On the second admission, eosinophilia (4,510/mm3) was pointed out. The case was diagnosed as hepatic vein obstruction by hepatic vein angiography, liver biopsy and ultrasonic examination. He had transient remission on corticosteroid and anti-coagulant therapy. This case was considered as a rare case of hypereosinophilic syndrome associated with pulmonary infarction and Budd-Chiari syndrome.     

Dressler's syndrome following pulmonary vein isolation for atrial fibrillation

Dressler's syndrome, characterized by features of fever, pericarditis and pericardial effusion typically occurs in the weeks to months following a myocardial infarction. The syndrome has also been described following several other myocardial and pericardial pathologies, including two reports of Dressler's syndrome following radio-frequency ablation. We describe a case of Dressler's syndrome following a pulmonary vein isolation procedure, which is being performed with increasing frequency as a treatment strategy for atrial fibrillation.     

Dressler's syndrome following pulmonary vein isolation for atrial fibrillation

Dressler's syndrome, characterized by features of fever, pericarditis and pericardial effusion typically occurs in the weeks to months following a myocardial infarction. The syndrome has also been described following several other myocardial and pericardial pathologies, including two reports of Dressler's syndrome following radio-frequency ablation. We describe a case of Dressler's syndrome following a pulmonary vein isolation procedure, which is being performed with increasing frequency as a treatment strategy for atrial fibrillation.     

Acute pulmonary embolism unmasking underlying chronic thromboembolic pulmonary hypertension and iliac vein compression syndrome

We describe the case of a 72‐year‐old gentleman who was referred to our institution for management of cardiogenic shock from a massive pulmonary embolism. Right heart catheterization revealed a low cardiac index and markedly elevated pulmonary pressures, suggested long‐standing venous thromboembolic (VTE) disease that evolved into chronic thromboembolic pulmonary hypertension (CTEPH). The patient was cannulated to veno‐arterial extra‐corporeal membrane oxygenation and eventually treated with pulmonary embolectomy and thromboendarterectomy. Subsequently discovered inferior vena cava clot and left iliac deep vein thrombosis were treated with suction and mechanical thrombectomy. Intravascular ultrasound of the left lower extremity venous system identified iliac vein compression syndrome (IVCS) as the culprit of the patient's VTE and CTEPH. A left iliac stent was placed and the patient was discharged on Warfarin for anticoagulation. The case illustrates the rapidly expanding armamentarium for VTE treatment and proposes IVCS as a new, potentially underrecognized risk factor for CTEPH.     

Natural histories of atrial septal defect with pulmonary hypertension, and ventricular septal defect with pulmonary hypertension

A study of the natural history of 51 adult patients with atrial septal defect with pulmonary hypertension (ASD + PH) was performed. ASD + PH of less than 14 Um2 of pulmonary artery resistance (PVR) was considered an indication for surgery. The prognosis of surgically treated patients was favorable, but that of medically treated patients was poor. For patients with ventricular septal defect with pulmonary hypertension (VSD + PH), surgery was considered for pulmonary-systemic vascular resistance ratio (Rp/Rs) less than 0.5, and for patients under than 10 years and, ideally, under 2 years of age.     

Different strategies for performing pulmonary vein isolation in patients with pulmonary vein rhythm

Pulmonary vein (PV) isolation was performed in atrial fibrillation (AF) patients whose cardiac rhythm was dominated by the ectopic beats originating from the PV. We herein report two cases with dominant PV ectopic rhythm that underwent catheter ablation for the treatment of paroxysmal AF. In one case, a permanent pacemaker implantation was required to treat a symptomatic long sinus pause after the isolation of all four PVs, while no AF was documented during the 5-year period after ablation. However, the isolation of all four PVs except for a PV with a dominant ectopic rhythm was performed in the other case. The latter case was free from both AF and symptomatic bradycardia following the procedure without the implantation of a pacemaker. Selective PV isolation therefore appears to be an effective therapy to both achieve the successful treatment of AF and to prevent the manifestation of sick sinus syndrome.