Malformaciones arteriovenosas pulmonares: presentación de un caso clínico pediátrico y revisión de la literatura / Pulmonary arteriovenous malformations: presentation of a pediatric case and review of the literature

Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

Bailout procedure in obstructed supracardiac total anomalous pulmonary vein drainage / Procedimiento de rescate en drenaje venoso pulmonar anómalo total supracardíaco obstructivo

Abstract Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the inter ventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

Resumen El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Mal-positioning of dialysis catheter in anomalous left superior pulmonary vein in a patient with acute type a dissection, a case report

Abstract The partial anomalous pulmonary vein drainage is a rare congenital defect. The pulmonary vein drains in to a systemic vein instead of draining in to the left atrium. In this rare birth defect, the right sided pulmonary vein involvement is more prevalent than the left sided pulmonary veins. We present a case where the anomalous left superior pulmonary vein was diagnosed when a renal dialysis catheter (size = 12F x 16cm) was mal-positioned in to the Anomalous left superior pulmonary vein, demonstrating confusing blood results. We describe how a systematic multidisciplinary approach and use of advanced imaging techniques can recognise and deal with this rare clinical dilemma.

Pulmonary vein parameters are similar or better predictors than left atrial diameter for paroxysmal atrial fibrillation after cryoablation

Left atrial diameter (LAD) has been considered an independent risk factor for atrial fibrillation (AF) relapse after pulmonary vein isolation (PVI). However, whether LAD or other factors are more predictive of late recurrence in patients with paroxysmal AF remains unclear. We aimed to evaluate the value of pulmonary vein (PV) parameters for predicting AF relapse 1 year after patients underwent cryoablation for paroxysmal AF. Ninety-seven patients with paroxysmal AF who underwent PVI successfully were included. PV parameters were measured through computed tomography scans prior to PVI. A total of 28 patients had recurrence of AF at one-year follow-up. The impact of several variables on recurrence was evaluated in multivariate analyses. LAD and the time from first diagnosis of AF to ablation maintained its significance in predicting the relapse of AF after relevant adjustments in multivariate analysis. When major diameter of right inferior pulmonary vein (RIPV) (net reclassification improvement (NRI) 0.179, CI=0.031-0.326, P<0.05) and cross-sectional area (CSA) of RIPV (NRI: 0.122, CI=0.004-0.240, P<0.05) entered the AF risk model separately, the added predictive capacity was large. The accuracy of the two parameters in predicting recurrence of AF were not inferior (AUC: 0.665 and 0.659, respectively) to echocardiographic LAD (AUC: 0.663). The inclusion of either RIPV major diameter or CSA of RIPV in the model increased the C-index (0.766 and 0.758, respectively). We concluded that major diameter of RIPV had predictive capacity similar to or even better than that of LAD for predicting AF relapse after cryoablation PVI.

Paroxysmal Atrial Fibrillation Catheter Ablation Outcome Depends on Pulmonary Veins Anatomy / O Resultado da Ablação por Cateter da Fibrilação Atrial Paroxística Depende da Anatomia das Veias Pulmonares

Abstract Background: Pulmonary veins (PV) are often the trigger to atrial fibrillation (AF). Occasionally, left PVs converge on a common trunk (LCT) providing a simpler structure for catheter ablation. Objective: To compare the clinical characteristics and outcomes of ablation in paroxysmal atrial fibrillation (PAF) of patients with or without LCT. Methods: Case-control study of patients undergoing first-ever catheter ablation procedure for drug refractory PAF. The information was taken from patients' records by means of a digital collection instrument, and indexed to an online database (Syscardio(r)). Clinical characteristics and procedures were compared between patients with or without LCT (LCT x n-LCT), adopting a level of statistical significance of 5%. The primary endpoint associated with efficacy was lack of atrial arrhythmia over the follow-up time. Results: One hundred and seventy two patients with PAF were included in the study, 30 (17%) LCT and 142 (83%) n-LCT. The clinical characteristics, comorbidities, symptoms scale and risk scores did not differ between the groups. There was AF recurrence in 27% of PAF patients in the n-LCT group and only 10% of patients in the LCT group (OR: 3.4 p: 0.04) after a follow-up of 34 ± 17 months and 26 ± 15 months respectively. Conclusion: Patients with a LCT have a significantly lower recurrence rate when compared to patients without this structure. It is mandatory to report the results of AF catheter ablation as a PV anatomical variation function.

Resumo Fundamento: As veias pulmonares (VP) são frequentemente o local de origem da fibrilação atrial (FA). Ocasionalmente, as VPs esquerdas confluem em um tronco comum (TrCE) proporcionando uma estrutura mais simples para ablação por cateter. Objetivos: Tem-se como objetivo comparar as características clínicas e os resultados da ablação em pacientes portadores de FA paroxística (FAP) com ou sem TrCE. Metódos: Estudo do tipo caso-controle de pacientes submetidos ao primeiro procedimento de ablação por cateter para FAP refratária a drogas antiarrítmicas. As informações foram retiradas dos prontuários dos pacientes por meio de instrumento digital de coleta e indexadas a uma base de dados online (Syscardio(r)). As características clínicas e procedimentos foram comparados entre pacientes com e sem TrCE (TrCE x n-TrCE), sendo adotado nível de significância estatística de 5%. O desfecho primário associado à eficácia foi ausência de arritmia atrial ao longo do seguimento com único procedimento. Resultados: Cento e setenta e dois pacientes foram incluídos no estudo, 30 (17%) TrCE e 142 (83%) n-TrCE. As características clínicas, comorbidades, severidade de sintomas e escores de risco não apresentaram diferença estatística entre os grupos. Houve recorrência da FA em 27% dos pacientes do grupo não-TrCE e em apenas 10% dos pacientes do grupo TrCE (OR: 3,4 p: 0.04) após um seguimento de 34±17 e 26±15 meses respectivamente. Conclusão: Pacientes com TrCE apresentam significativamente menor taxa de recorrência quando comparados a pacientes sem esta estrutura. É imprescindível relatar os resultados da ablação por cateter de FA na vigência de variações anatômicas das VPs.

Pulmonary Vein Anatomy is Associated with Cryo Kinetics during Cryoballoon Ablation for Atrial Fibrillation / A Anatomia da Veia Pulmonar está Associada à Criocinética durante a Ablação por Criobalão da Fibrilação Atrial

Abstract Background: The influence of pulmonary vein (PV) anatomy on cryo kinetics during cryoballoon (CB) ablation is unclear. Objective: To investigate the relationship between PV anatomy and cryo kinetics during CB ablation for atrial fibrillation (AF). Methods: Sixty consecutive patients were enrolled. PV anatomy, including ostial diameters (long, short and corrected), ratio between short and long diameters, ostium shape (round, oval, triangular, and narrow), and drainage pattern (typical, with common trunk, common antrum, ostial branch and supernumerary PV) were evaluated on multi-detector computed tomography (MDCT) images pre-procedure. Cryo kinetics parameters [balloon freeze time from 0 to -30ºC (BFT), balloon nadir temperature (BNT) and balloon warming time from -30 to +15ºC (BWT)] were recorded during procedure. All p values are two-sided, with values of p < 0.05 considered to be statistically significant. Results: 606 times of freezing cycle were accomplished. Moderate negative correlation was documented between BNT and corrected PV diameter (r = -0.51, p < 0.001) when using 23-mm CBs, and mild negative correlation (r = - 0.32, p = 0.001) was found when using 28-mm CBs. Multivariate logistic regression analysis revealed that PV corrected ostial diameter (OR, 1.4; p = 0.004) predicted a BNT < -51ºC when using 23-mm CBs, while PV ostium oval shape (OR, 0.3; p = 0.033) and PV locations (left inferior PV: OR, 0.04; p = 0.005; right superior PV: OR, 4.3; p = 0.025) predicted BNT < -51ºC when using 28-mm CBs. Conclusions: MDCT can provide PV anatomy accurate evaluation prior CB ablation. PV anatomy is associated with cryo kinetics during ablation.

Resumo Fundamentos: A influência da anatomia da veia pulmonar (VP) na criocinética durante a ablação por criobalão (CB) não está clara. Objetivo: Investigar a relação entre a anatomia da VP e a criocinética durante a ablação com CB para fibrilação atrial (FA). Métodos: sessenta pacientes consecutivos foram matriculados. Foram avaliados em imagens de tomografia computadorizada multidetectora (TCMD) pré-procedimento a anatomia da VP, incluindo diâmetros dos óstios (longo, curto e corrigido), relação entre diâmetros curtos e longos, forma do óstio (redondo, oval, triangular e estreito) e padrão de drenagem (típico, com tronco comum, antro comum, ramo ostial e VP supranumerária). Os parâmetros criocinéticos [tempo de congelamento de balão de 0 a -30ºC (TCB), temperatura do nadir do balão (TNB) e tempo de aquecimento do balão de -30 a + 15ºC (TAB)] foram registrados durante o procedimento. Todos os valores de p são bicaudais, com valores de p < 0,05 considerados estatisticamente significativos. Resultados: o ciclo de congelamento foi realizado 606 vezes. Correlação negativa moderada foi documentada entre o TNB e o diâmetro VP corrigido (r = - 0,51, p < 0,001) ao usar CBs de 23 mm e correlação negativa leve (r = - 0,32, p = 0,001) foi encontrada ao usar 28- mm CBs. A análise de regressão logística multivariada revelou que o diâmetro corrigido do óstio da VP (OR, 1,4; p = 0,004) previu um TNB < -51ºC ao usar CB de 23 mm, enquanto a forma oval do óstio VP (OR, 0,3; p = 0,033) e as localizações da VP (VP inferior: OR, 0,04; p = 0,005; VP superior direito: OR, 4,3; p = 0,025) previram TNB < -51ºC ao usar CBs de 28 mm. Conclusões: A TCMD pode fornecer uma avaliação precisa da anatomia da VP antes da ablação por CB. A anatomia da VP está associada à criocinética durante a ablação.

Velocity time integral for right upper pulmonary vein in VLBW infants with patent ductus arteriosus

OBJECTIVE: Early diagnosis of significant patent ductus arteriosus reduces the risk of clinical worsening in very low birth weight infants. Echocardiographic patent ductus arteriosus shunt flow pattern can be used to predict significant patent ductus arteriosus. Pulmonary venous flow, expressed as vein velocity time integral, is correlated to ductus arteriosus closure. The aim of this study is to investigate the relationship between significant reductions in vein velocity time integral and non-significant patent ductus arteriosus in the first week of life. METHODS: A multicenter, prospective, observational study was conducted to evaluate very low birth weight infants (<1500 g) on respiratory support. Echocardiography was used to evaluate vein velocity time integral on days 1 and 4 of life. The relationship between vein velocity time integral and other parameters was studied. RESULTS: In total, 98 very low birth weight infants on respiratory support were studied. On day 1 of life, vein velocity time integral was similar in patients with open or closed ductus. The mean vein velocity time integral significantly reduced in the first four days of life. On the fourth day of life, there was less of a reduction in patients with patent ductus compared to those with closed patent ductus arteriosus and the difference was significant. CONCLUSIONS: A significant reduction in vein velocity time integral in the first days of life is associated with ductus closure. This parameter correlates well with other echocardiographic parameters and may aid in the diagnosis and management of patent ductus arteriosus.

Signo de la cimitarra / Scimitar sign

The scimitar sign refers to the image resembling a short, curved Turkish sword called scimitar, created by an anomalous pulmonary vein. It can be seen in chest radiography, computed tomography and magnetic resonance. It forms part of the scimitar syndrome, characterized by partial or total anomalous pulmonary venous drainage, associated with hypoplasia of the right lung and dextrocardia. Depending on the magnitude of the right-to-left shunt, it may present in children with pulmonary hypertension and right cardiac failure, or as asymptomatic imaging findings in adults.

El signo de la cimitarra es la imagen creada por una vena pulmonar anómala similar a una espada turca, corta y curva llamada cimitarra. Se puede ver en radiografía de tórax, tomografía computada y resonancia magnética. Forma parte del síndrome de la cimitarra, caracterizado por un drenaje pulmonar anómalo, parcial o total, asociado a hipoplasia del pulmón derecho y dextrocardia. Dependiendo de la magnitud del shunt de derecha a izquierda producido, se puede presentar en edades infantiles con hipertensión pulmonar y falla cardiaca derecha, o en adultos, de manera asintomática, como hallazgo imagenológico.

Pulmonary varices - rare cause of haemoptysis

Haemoptysis has a long list of causes, but pulmonary varices are amongst the rare causes. Pulmonary varices are rare abnormalities of pulmonary veins that may exist either as an isolated malformation or in association with pulmonary venous hypertension. This case report describes pulmonary varices as a cause of hemoptysis in an adolescent boy, which was diagnosed on multislice CT and confirmed on angiography

Individual Pulmonary Vein Atresia in Adults: Report of Two Cases

We present two cases of individual pulmonary vein atresia without vestige of an involved pulmonary vein. On CT, we noted the absence or interruption of normal pulmonary venous structures, and the presence of abnormal vascular structures that represented collaterals for the involved lung parenchyma. On angiography, the atretic pulmonary vein was found to drain into the other ipsilateral pulmonary veins through the collaterals.

Infracardiac total anomalous pulmonary venous drainage with unusual presentation.

Total anomalous pulmonary venous drainage (TAPVD) is a rare entity which forms approximately 0.4 to 2% of all congenital heart disease. The infracardiac type usually involve obstructions on pulmonary venous connections and comprising a quarter of all TAPVD cases. The clinical findings in patients with obstructed infracardiac TAPVD could mimic respiratuary distress of several different etiologies during first hours of life. In this article,we present a case of a neonate with infracardiac type of TAPVD presented with only distinct subcutaneous veins of abdominal and thoracic wall.

Embolotherapy for Pulmonary Arteriovenous Malformations in Patients without Hereditary Hemorrhagic Telangiectasia

OBJECTIVE: To evaluate the clinical and radiological outcomes of transcatheter embolotherapy for treating sporadic pulmonary arteriovenous malformations (PAVMs) that were not associated with hereditary hemorrhagic telangiectasia. MATERIALS AND METHODS: Between January 2001 and June 2008, thirty-five sporadic PAVMs were detected in 23 patients. The clinical follow up consisted of assessing the changes of the signs and symptoms of the PAVMs, and radiological evaluation with chest radiographs or chest CT scans. RESULTS: The lower lung regions (63%) and peripheral locations (86%) were the common locations of the PAVMs. Thirty-four PAVMs (97%) had simple architecture (one arterial feeder within a single pulmonary segment). Technical success was achieved in 33 PAVMs (94%); two cases of technical failure were due to catheterization failure (n = 1) and too large a feeding artery (17 mm) that disabled embolotherapy (n = 1). Coils and Amplatz vascular plugs were used in 30 and three PAVMs, respectively. Inadvertent placement of one coil (n = 1) and pulmonary infarction (n = 1) occurred, but no relevant symptoms developed. For the 13 patients with available data, the mean arterial O2 saturation changed significantly from 92% to 98%. Complete or near-complete involution of the sac was observed in 30 of the 33 embolized PAVMs (91%). In these 33 embolized PAVMs, the mean sac diameter significantly decreased from 17.83 mm to 0.68 mm. CONCLUSION: Sporadic PAVMs are mostly the simple type with predominance in the lower lobe and peripheral locations. Transcatheter embolotherapy with coils or Amplatz vascular plugs is a safe and effective treatment for sporadic PAVMs and this provides excellent functional and radiological improvement.

Quantification of AS and AR.

Trans-esophageal echocardiography (TEE) is routinely used in valvular surgery in most institutions. The popularity of TEE stems from the fact that it can supplement or confirm information gained from other methods of evaluation or make completely independant diagnoses. Quantitative and qualitative assessment permits informed decisions regarding surgical intervention, type of intervention, correction of inadequate surgical repair and re-operation for complications. This review summarizes the various methods for quantification of aortic regurgitation and stenosis on TEE. The application of Doppler echo (pulsed wave, continuous wave and color) with two-dimensional echo allows the complete evaluation of AV lesions.

Virtual CT Colonoscopy and Virtual CT Barium Enema using Multidetector-row CT / 대한소화기학회지

CT colonography is a promising technique that provides both multiplanar and endoluminal perspective of the air-filled, distended, cleaned colon. "Virtual colonoscopy" refers to computer-simulated 3D endoscopic visualization of the colonic mucosal surface. Unlike barium enema and conventional colonoscopy, CT colonography can give cross- sectional and endoluminal images of the colon and enables to image extracolic abnormality. CT colonography offers potential advantages over colonoscopy in that it causes little discomfort to the patient, and does not need sedation. It is more accurate in spatial location of lesions and creates no complication. To date, most studies assessing CT colonography have focused in technical development, less aggressive bowel preparation, and computer-aided diagnosis of polyp detection. In the future, CT colonography would be a diagnostic and screening tool for the colorectal polyp and cancer.