Precision diagnosis and therapy of a case of brain abscesses associated with asymptomatic pulmonary arteriovenous fistulas.

BACKGROUND: Brain abscesses, a severe infectious disease of the CNS, are usually caused by a variety of different pathogens, which include Streptococcus intermedius (S. intermedius). Pulmonary arteriovenous fistulas (PAVFs), characterized by abnormal direct communication between pulmonary artery and vein, are a rare underlying cause of brain abscesses. CASE PRESENTATION: The patient was a previous healthy 55-year-old man who presented with 5 days of headache and fever. Cerebral magnetic resonance imaging (MRI) suggested a brain abscess. Thoracic CT scan and angiography demonstrated PAVFs. Aiding by metagenomic next-generation sequencing (mNGS) of the cerebrospinal fluid (CSF) sample which identified S. intermedius as the causative pathogen, the patient was switched to the single therapy of large dose of penicillin G and was cured precisely and economically. CONCLUSIONS: It is an alternative way to perform mNGS to identify causative pathogens in patients with brain abscesses especially when the results of traditional bacterial culture were negative. Further thoracic CT or pulmonary angiography should also be undertaken to rule out PAVFs as the potential cause of brain abscess if the patient without any known premorbid history.

A Rare Case of Cerebral Air Embolism Caused by Pulmonary Arteriovenous Malformation After Removal of a Central Venous Catheter.

Cerebral air embolism following central venous catheter (CVC) removal is extremely rare. We report a case of cerebral air embolism with loss of consciousness after removal of CVC caused by pulmonary arteriovenous malformation (PAVM). Computed tomography revealed air bubbles in the internal carotid arteries along the sulci in the cerebral hemispheres, as well as a PAVM. The cerebral air embolism was treated with hyperbaric oxygen and intravenous thrombolytic therapy, and transcatheter embolization of the PAVM was performed. When inserting/removing CVC in a patient with a small PAVM, treatment of the PAVM, irrespective of its size, could prevent the type of complication that occurred in our present case.

Mapping and ablation of anteroseptal atrial tachycardia in patients with congenitally corrected transposition of the great arteries: implication of pulmonary sinus cusps.

AIMS: We sought to investigate focal atrial tachycardias (ATs) in patients with congenitally corrected transposition of the great arteries (ccTGA). METHODS AND RESULTS: We identified three cases of focal ATs with ccTGA from 2007 to 2015. The clinical findings, electrocardiography, electrophysiological features, and ablation were reported. All three cases had {S,L,L}-type ccTGA. The AT P waves shared the following features: narrow in width, positive in leads I and aVL, and negative/positive in lead V1. All ATs had focal activation pattern with early activation at the anteroseptal area, and further mapping demonstrated earliest atrial activation (EAA) inside the pulmonary sinus cusps (PSCs), especially the right PSC. In Cases 1 and 2, double potentials were recorded at the EAA sites. Conduction delay between the two potentials during atrial extrastimulus was observed. After successful ablation, the second potential during sinus rhythm was further delayed in Case 1 and was eliminated in Case 2. However, only one potential was recorded in Case 3. All three cases were successfully managed by ablating the sites with EAA, which preceded the P-wave onset by 60, 80, and 42 ms, respectively. During ablation, no junctional rhythm was observed. Incomplete left bundle branch block was induced during mapping in Case 1, but no other complications occurred. All patients were free from tachycardia during follow-up periods of 36, 7, and 5 months, respectively. CONCLUSION: Anteroseptal AT in patients with ccTGA could be eliminated by ablation inside the PSC.

Spontaneous cerebral gas embolism and pulmonary arteriovenous malformation: a case report.

Pulmonary barotrauma can cause cerebral arterial gas embolism (CAGE) from pulmonary overdistension of alveoli forcing gas into the pulmonary vasculature. We report a case of CAGE in a man found to have occult pulmonary arteriovenous malformation (PAVM) and undiagnosed obstructive sleep apnea (OSA). A 46-year-old man was admitted to the hospital for an acute seizure and left-sided weakness, with telangiectasias on his lower lip and tongue. Brain-computed tomography (CT) showed gas emboli in the right hemisphere. Chest CT revealed a 1.8-cm PAVM in the posterior right costophrenic sulcus. A transthoracic echocardiogram showed no intracardiac shunt or patent foramen ovale. He was treated with phenytoin, lidocaine and hyperbaric oxygen. The PAVM was occluded with a detachable balloon followed by coil embolization. Polysomnography revealed severe obstructive sleep apnea, which was treated with CPAP. Seven years later, the patient was functioning at his pre-event baseline. We propose the CAGE was caused by high negative intrathoracic pressures while breathing against an obstructed upper airway, with air entrainment into the PAVM and subsequent arterialization.

Cerebral iodized lipid embolization via a pulmonary arteriovenous shunt: rare complication of transcatheter arterial embolization for hepatocellular carcinoma.

We report the first European case of cerebral iodized lipid embolism post transcatheter arterial embolization for hepatocellular carcinoma. Lipiodol emboli and corresponding multifocal brain ischemia were documented with computed tomography (CT) and magnetic resonance (MR) in the acutely symptomatic patient. Transcranial Doppler sonography with contrast indicated a right-to-left shunt, while on a follow-up CT scan lipiodol embolization was detected in both lungs. Dilated pulmonary vessels and thick vascular channels were seen in the vicinity of the right diaphragm suggestive of pulmonary arteriovenous shunt. The patient symptoms regressed with supportive care alone, but he died 5 months later due to hepatic failure unrelated to the procedure.

Landiolol for junctional ectopic tachycardia refractory to amiodarone after pediatric cardiac surgery.

Postoperative junctional ectopic tachycardia (JET) in children undergoing cardiac surgery is a serious arrhythmia that is associated with considerable morbidity and mortality. We present here a case of successful landiolol therapy for postoperative JET in a 3-month-old infant who underwent ventricular septal defect closure and right pulmonary artery plasty. His left ventricular function was poor postoperatively. The JET was refractory to amiodarone and caused severe hypotension, which was required cardiac massage. Continuous intravenous infusion of low-dose landiolol reduced the persistent JET rate immediately, and restored to sinus rhythm with stable hemodynamics.

Persistence of bilateral arterial ducts in pulmonary atresia despite confluent branch pulmonary arteries: opportunity for two percutaneous therapeutic alternatives.

We present a case of a newborn infant with double inlet left ventricle, pulmonary atresia, confluent pulmonary arteries, and bilateral arterial ducts (AD), to discuss the therapeutic alternatives offered by interventional catheterization techniques in this anatomic arrangement. The infant initially underwent stenting of the right AD to stabilize pulmonary blood flow off of prostaglandin infusion. Three weeks later, she developed left pulmonary artery isolation upon closure of the left arterial duct. An additional stent was placed in the pulmonary artery confluence, restoring blood flow to the left lung and significantly improving her oxygen saturations. At 6 months of age she underwent her first surgical procedure, a successful bidirectional cavopulmonary anastomosis with removal of the left pulmonary artery stent and patch enlargement of the pulmonary artery confluence. She continues to do well in clinical follow-up at 16 months of age.

Clinical application of tissue engineered human heart valves using autologous progenitor cells.

BACKGROUND: Tissue engineering (TE) of heart valves reseeded with autologous cells has been successfully performed in vitro. Here, we report our first clinical implantation of pulmonary heart valves (PV) engineered with autologous endothelial progenitor cells (EPCs) and the results of 3.5 years of follow-up. METHODS AND RESULTS: Human PV allografts were decellularized (Trypsin/EDTA) and resulting scaffolds reseeded with peripheral mononuclear cells isolated from human blood. Positive stain for von Willebrand factor, CD31, and Flk-1 was observed in monolayers of cells cultivated and differentiated on the luminal surface of the scaffolds in a dynamic bioreactor system for up to 21 days, indicating endothelial nature. PV reseeded with autologous cells were implanted into 2 pediatric patients (age 13 and 11) with congenital PV failure. Postoperatively, a mild pulmonary regurgitation was documented in both children. Based on regular echocardiographic investigations, hemodynamic parameters and cardiac morphology changed in 3.5 years as follows: increase of the PV annulus diameter (18 to 22.5 mm and 22 to 26 mm, respectively), decrease of valve regurgitation (trivial/mild and trivial, respectively), decrease (16 to 9 mm Hg) or a increase (8 to 9.5 mm Hg) of mean transvalvular gradient, remained 26 mm or decreased (32 to 28 mm) right-ventricular end-diastolic diameter. The body surface area increased (1.07 to 1.42 m2 and 1.07 to 1.46 m2, respectively). No signs of valve degeneration were observed in both patients. CONCLUSIONS: TE of human heart valves using autologous EPC is a feasible and safe method for pulmonary valve replacement. TE valves have the potential to remodel and grow accordingly to the somatic growth of the child.

Bronchial artery aneurysm embolization with NBCA.

We present a case of asymptomatic bronchial artery aneurysm that formed a fistula with part of the pulmonary artery (there was no definite fistula with the pulmonary vein). We were able to catheterize the feeding vessel but could not reach the aneurysm. We therefore injected a mixture of N-butyl-2-cyanoacrylate (NBCA; Histoacryl, B. Braun, Melsungen, Germany) and iodized oil (Lipiodol; Guerbet, Aulnay-sous-Bois, France) from the feeding vessel. The fistula, aneurysm, and feeding vessel were almost totally occluded. After embolization, the patient coughed a little; there were no other definite side effects or complications. One and 3 months later, on chest CT, the aneurysm was almost completely occupied with hyperattenuating NBCA-Lipiodol embolization. NBCA is a liquid embolization material whose time to coagulation after injection can be controlled by diluting it with Lipiodol. It is therefore possible to embolize an aneurysm, feeding vessels, and efferent vessels (in our case, it was a fistula) by using an NBCA-Lipiodol mixture of an appropriate concentration, regardless of whether the catheter can reach the aneurysm or not.

Severe bilateral panlobular emphysema and pulmonary arterial hypoplasia: unusual manifestations of Menkes disease.

Menkes disease is an X-linked recessive disorder of copper transport characterized by neurological deterioration, connective tissue, and vascular defects, abnormal hair, and death in early childhood. We report on a patient with Menkes disease in whom severe diffuse emphysema caused respiratory failure and death at 14 months of age. He had severe growth and developmental delays and other typical clinical manifestations of Menkes disease. He developed respiratory problems requiring continuous supplemental oxygen and a progressively enlarging soft tissue mass appeared on the neck. Imaging studies revealed cystic spaces in multiple lobes of the lung consistent with bullous emphysema. The neck mass was determined to be an internal jugular venous aneurysm. At autopsy, extensive emphysematous change was evident. Post-mortem barium injections of the pulmonary arterial system revealed marked dilatation and tortuosity of the preacinar pulmonary arteries and reduced numbers of intra-acinar arteries. Severe emphysema, presumably caused by abnormal elastin due to deficiency of the copper-dependent enzyme lysyl oxidase, may represent an underestimated clinical complication of Menkes disease and should be considered in the differential diagnosis of chronic respiratory disease in these patients.

[Diagnostic considerations in unilateral hyperlucency of the lung (Swyer-James-MacLeod Syndrome)]. / Consideraciones diagnósticas sobre el llamado síndrome del pulmón hiperclaro unilateral (síndrome de Swyer-James o de Mc-Leod).

Swyer-James-MacLeod Syndrome (SJMS) is considered to be a relatively uncommon and complex disease characterized by roentgenographic hyperlucency of one lung, lobe, or part of a lobe, due the pulmonary vascular structure and alveolar overdistension. It is sometimes associated with bronchiectasis. This syndrome seems to be an acquired disease that develops after viral bronchiolitis and/or viral pneumonia in early childhood. Microscopically, there is evidence of patchy bronchitis and bronchiolitis.SJMS is usually asymptomatic and discovered accidentally by chest radiography in a child with respiratory symptoms and should be differentiated from other causes of unilateral hyperlucency on chest radiography, such as those related to congenital bronchial and/or vascular abnormalities. Treatment includes early control of lung infections, as well as influenza and pneumococcal vaccination. Few reports of this syndrome in children have been published. We describe the case of a 12-year-old boy with unilateral hyperlucency of the lung and respiratory symptoms of acute pneumonia and discuss the main diagnostic features of this syndrome.

Occurrence and management of atrial arrhythmia after long-term Fontan circulation.

OBJECTIVES: In patients after the Fontan operation, we determined risk factors for late failure and for intra-atrial re-entrant tachycardia at 15 to 20 years' follow-up. Midterm results after electrophysiologic ablation therapy for these tachycardias were also evaluated. METHODS: Current follow-up was available in 162 patients (2005 patient-years) with a wide range of underlying diagnoses operated on between February 1978 and May 1995. Risk factor analysis included patient-related and procedure-related variables, with late failure and the incidence of re-entrant tachycardia as outcome parameters. RESULTS: Forty late failures were observed (2.0 per 100 patient-years). At 15 years, Kaplan-Meier estimated survival was significantly (P =.007) better for patients with tricuspid atresia (93%) compared with that for patients with complex congenital malformation (71%). The sole multivariable risk factor for Fontan failure was the type of underlying diagnosis. At 20 years' follow-up, overall freedom from tachycardia was estimated to be 46% +/- 12%. Acute success of electrophysiologic ablation was seen in 25 (83%) of 30 patients, and Kaplan-Meier estimated freedom from recurrent tachycardia was 81% +/- 10% at 3 years. Multivariate analysis identified duration of Fontan circulation as the sole risk factor for re-entrant tachycardias. CONCLUSION: After the modified Fontan operation, long-term survival in patients with tricuspid atresia was significantly better compared with that in patients with complex congenital malformations. As first-choice therapy for atrial re-entrant tachycardias, we recommend electrophysiologic ablation therapy.

Pulmonary structural maturation and pulmonary artery remodeling after reversible fetal ovine tracheal occlusion in diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with thickened pulmonary arteries (PA) contributing to pulmonary hypertension. In the current study, the effects of antenatal glucocorticoids and reversible tracheal occlusion (TO) on PA structure were assessed in a hypoplastic lung model. METHODS: A left-sided CDH was created in fetal lambs at 80 days gestation, TO at 108 days, and release of the occlusion (TR) at 129 days. All were given 1 dose of maternal glucocorticoids at 135 days. At 136 days (term, 145 days), the fetus was delivered by cesarian section. CDH (n = 7), CDH + TO (n = 6), CDH + TO + TR (n = 6), and unoperated twin controls (n = 16) were compared. Outcome measurements were (1) lung growth, represented by lung weight to body weight ratio (LW/BW), (2) lung structural maturation, which is inversely proportional to mean terminal bronchiole density (MTBD), (3) PA medial and adventitial areas (square micrometers), (4) lung capillary load, which is the ratio of vessel surface area (SA) to tissue SA ratio. RESULTS: CDH lungs were hypoplastic with a low LW/BW and high MTBD. The small PAs (<75 microm) of CDH had an increased medial area, indicating increased muscle mass and an increased adventitial area. CDH + TO +/- TR increased LW/BW and achieved normal structural lung maturity with a low MTBD. Only CDH + TO thinned the PA medial area closer to control values. The adventitial area remained thick in CDH +/- TO +/- TR when compared with controls. All 4 groups had similar capillary load. CONCLUSIONS: TO may be especially important for PA remodeling in the latter part of gestation, because TR 1 week before delivery prevents thinning of the small PAs in CDH. The shaping achieved by TO in terms of lung growth, structural maturity, and pulmonary artery medial area thinning may prove beneficial in lessening the severity of the associated pulmonary hypertension in CDH.

[Surgical treatment of atrial septal defects and associated congenital heart diseases]. / Khirurgicheskoe lechenie pri defekte mezhpredserdnoi peregorodki i sochetaiushchikhsia s nim porokakh serdtsa.

The results of radical correction of interatrial septum defects and heart diseases associated with it were analyzed after treatment of 502 patients at the age from 5 to 48 years. In most cases (58.4%) the patients were adults. The isolated defect of the interatrial septum was diagnosed in 377 patients. Correction of the defect was performed in 82 patients (16.3%) under mild hypothermia and 420 patients (83.6%) were operated upon under conditions of artificial circulation. General lethality was 7.7%.

[Pulmonary arterial hypertension in patients with hereditary hemorrhagic telangiectasia (Osler-Rendu disease)]. / Legochnaia arterial'naia gipertenziia u bol'nykh nasledstvennoi gemorragicheskoi teleangiektaziei (bolezn'iu Randiu-Oslera).

Pulmonary arterial pressure was studied using a noninvasive method in 23 patients with hereditary hemorrhagic telangiectasia (HHT) without clinical and x-ray signs of arterial-venous shunting in the lungs. It was established that pulmonary arterial hypertension was typical of HHT patients. A significant decrease in the volume and frequency of hemorrhages in 12 of 17 HHT patients was combined with a statistically significant decrease in pulmonary arterial pressure as a result of HBO therapy. It was assumed that pulmonary microcirculatory disorders played an important role in HHT pathogenesis and were expressed in metabolic derangement of a number of biologically active substances in the lungs resulting in the development of telangiectasia.