Resolution of Secondary Pulmonary Arteriovenous Malformations after Embolization of a Congenital Superior-Mesenteric-Vein-to Left-Renal-Vein Shunt (original) (raw)
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Journal of Clinical Medicine
Pulmonary arteriovenous malformations (PAVMs) are uncommon, predominantly congenital direct fistulous connections between the pulmonary arteries and pulmonary veins, resulting in a right to left shunt. Patients with PAVMs are usually asymptomatic with lesions detected incidentally when radiological imaging is performed for other indications. In this review, we discuss the classification and radiological features of PAVMs as well as their treatment and follow-up options, with a particular focus on percutaneous endovascular techniques and the evolution of the available equipment for treatment.
Pulmonary Arteriovenous Malformations in a 18-months-old Child: A Case Report
JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH
Pulmonary Arteriovenous Malformation (PAVM) is a cardiovascular anomaly in which an anatomic communication between the pulmonary artery and pulmonary vein is formed, resulting in an additional cardiac shunt from the right to left side. PAVM is a rare disease, reported more in females, but in newborns comparatively higher in males. Cases can vary from asymptomatic to symptomatic such as having dyspnoea, hypoxia, cyanosis, neurological symptoms. They are mostly congenital having association with Hereditary Haemorrhagic Telangiectasia (HHT). Herby, author present a case of 18-months-old female was admitted with peripheral cyanosis in the Paediatric Department. Her saturation was 76%, after admission she received three doses of salbutamol nebulisation and was kept on 3 litre oxygen until her saturation increased to around 90%. On auscultation, a murmur of grade 2/6 heard in the left axilla with clear lung fields. Chest X-ray detected a prominent lobulated opacity in the left sided lung ...
Journal of International Medical Research, 2005
Pulmonary arteriovenous malformations (PAVMs) are rare anomalies. The degree of right-to-left shunting, which can lead to cyanosis and paradoxical embolism causing neurological complications, determines the prognosis. We report two cases of PAVM and review the literature. A 45-year-old woman with clinical signs and symptoms of PAVM was examined using several different scanning techniques, which showed a large PAVM in the lower lobe of her right lung. A lobectomy was performed, which revealed a 5 cm diameter PAVM with one feeding artery and multiple veins. Intravenous angiography of a 7-year-old girl with symptoms of fatigue and acro-cyanosis confirmed the presence of a large PAVM in her right lower lobe. The PAVM had two major arteries arising from the aorta, which were ligated during a lobectomy. Both patients recovered well following surgery. Although PAVMs are rare, their neurological and haemodynamic consequences may be fatal. Interventional treatment techniques, including surge...
Revision of facts on pulmonary arteriovenous malformations – experience with embolization techniques
2017
Introduction. Pulmonary arteriovenous malformations are pathological direct connections between arterial and venous circulation, bypassing capillary networks. The consequences of the occurring right-to-left shunt comprise systemic hypoxemia and loss of pulmonary vascular filtration properties, which pose a risk of serious life-threatening neurologic complications because of paradoxical embolism. 60-90% of PAVMs appear to be a manifestation of the underlying Hemorrhagic Hereditary Telangiectasia. Treatment is introduced as protective measures against life-threatening hemorrhagic or neurologic symptoms, and presently transcatheter embolization proved to be a method of choice. Aim. The aim of this study was to discuss the efficacy and technical details of the selected current approaches to PAVM treatment with respect to the angioarchitecture of the fistulae, based on the literature review and clinical experience. Material and methods. We performed a retrospective investigation of 5 pat...
The Turkish Journal of Pediatrics
A 5-month-old boy with progressive respiratory distress was admitted to our hospital. Physical examination revealed mild tachypnea and retraction. The left main bronchus was found as severely collapsed between the right pulmonary artery and the descending aorta, on the bronchoscopic evaluation. Further evaluation revealed persistent ductus venosus (PDV). As in the fetal period the ductus venosus arises from the posterior aspect of the left portal vein, a PDV is considered another type of intrahepatic portosystemic shunt. Therefore, he was diagnosed with congenital portosystemic venous shunt, leading to persistent respiratory distress. The PDV was closed with Amplatzer vascular plug II, and then he had immediate clinical improvement. Congenital portosystemic venous shunts (CPSS) are rare vascular malformations associated with severe complications. Here we presented a case with progressive respiratory distress as a result of CPSS and rapid improvement after embolization.
A case report on an incidental discovery of congenital portosystemic shunt
Medicine, 2019
Rationale: Congenital portosystemic shunt (Abernethy malformation) is a rare entity causing the portal blood to drain directly into the systemic circulation, eluding the liver. These shunts arise through disturbances in the embryonic development. Patient concerns: A 9-year-old male patient was referred to our department for further evaluation of a vascular malformation which was diagnosed in another facility when the patient was 2 years old, following a routine abdominal ultrasound. The patient had no complaints and the physical examination was normal at all times. Diagnosis: Laboratory tests and esogastroduodenoscopy were normal. The abdominal ultrasound showed a side-to-side shunt between a short portal trunk and the inferior vena cava. A hepatic mass suggestive for focal noduar hyperplasia was seen in the left liver lobe. Abdominal angio-computed tomography (angio-CT) was performed and the ultrasonographic anomalies were confirmed. Multiple other vascular malformations were diagnosed-hepatic artery emerging from the superior mesenteric artery, with early division; hepatic veins forming a short common trunk before draining into the inferior vena cava; supranumerary right renal artery emerging from the aorta, tributary for the upper renal pole. Ecocardiography showed left superior vena cava persistence. The final diagnosis was Abernathy malformation type IB. In the meantime the patient was diagnosed with allergic asthma. Interventions: No surgical cure was pursued because the malformation was an incidental discovery. Outcomes: The patient was followed-up closely from the final diagnosis (when he was 9 years old) to present (he is currently 10 years old) with no change in his status-he remained asymptomatic. Lessons: Angio-CT should be the performed whenever a vascular malformation is suspected in order to establish a correct diagnosis, because portosystemic shunts carry a high risk of severe complications. Knowing that patients with portosystemic shunts may have pulmonary hypertension, respiratory complaints should be carefully evaluated-in this particular case, even though the most probable cause for the respiratory symptoms was pulmonary hypertension, it was ruled out by cardiac ultrasonography and further investigations confirmed the diagnosis of allergic asthma. Abbreviations: angio-CT = computed tomography angiography, cm = centimeter, CT = computed tomography, i.v. = intravenous, sec = seconds, VRT = volume rendering technique, MIP = maximum intensity projection.
The Annals of Thoracic Surgery, 2008
1. Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 1978; 41:2250 -66. 2. Korbmacher B, Doering C, Schulte HD, et al. Malignant fibrous histiocytoma of the heart: case report of a rare left-atrial tumor. Thorac Cardiovasc Surg 1992; 40:303-7. 3. Okamoto K, Kato S, Katsuki S, et al. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Intern Med 2001; 40:1222-6. 4. Dorobantu M, Fruntelate A, Constantinescu D, et al. Primary left heart malignant fibrous histiocytoma. Eur J Echocardiogr 2005; 6:225-7. 5. Fujio A, Kitano M, Asakura S, Matsui T. A case of malignant histiocytoma of the epicardium. Nippon Kyobu Geka Gakkai Zasshi 1986; 34:267-71. 6. Wilk W, Janicki K. Primary intrapericardial malignant fibrous histiocytoma: a case report. Pol J Pathol 1998; 49:43-6. 7. Tanprasert P, Vidhayasarronyut S, Dharamadhach A, Suchatlampong V. A rare case of pericardial malignant fibrous histiocytoma. J Med Assoc Thai Comment Pulmonary arteriovenous malformation is a hereditary disease; approximately 70% of patients have hereditary hemorrhagic telangiectasia [3]. Conversly, 15% to 35% of the patients with hereditary hemorrhagic telangiectasia have PAVMs [3]. Pulmonary arteriovenous malformation occurs twice as often in females as in males, but there is a male predominance in newborns [4].
Operation: the Systemic-Pulmonary Artery Shunt
2016
Objective-The true incidence of congenital cardiovascular malformations is difficult to determine accurately, partly because of difficulties in definition. About 0.8 percent of live births are complicated by a cardiovascular malformation. Hypoxia and cyanosis, the common complications of all cyanotic disease, may be life-threatening in severe forms. Today, the trend is towards the total surgical correction of these anomalies in early life. As the accomplishment of this strategy in various parts of the world is not possible, palliative procedures like systemic-pulmonary shunt have retained their importance. Methods-Data were collected from the files of 180 patients, for whom systemic-pulmonary shunt was performed by a single surgical group at our center between March 1992 and May 2006. Our aim was to determine the outcome of shunt operation in terms of success rate, morbidity, and mortality. Results-The median age and weight of the patients was 24 months and 10.5 kilograms, respectively. There was a spectrum of underlying cyanotic heart diseases. The main operation was the modified Blalock-Taussig shunt (90%). The mean value of oxygen saturation was 62% pre-operatively, which rose to 85% after surgery. We found a 77.9% success rate, 6.7% mortality rate, and 8.7% morbidity rate. Conclusion-There was no significant correlation between the predictive factors and success of operation. Lower age and weight of the patient, small size of the pulmonary artery, and urgency of operation predicted the operative mortality