Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant.

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.

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.

Pulmonary Arteriography in Pulmonary Vein Stenosis.

A 43-year-old woman underwent radiofrequency pulmonary vein ablation for symptomatic paroxysmal atrial fibrillation. At 3 months, she developed worsening dyspnea and exercise intolerance; tests revealed severe stenosis in her right pulmonary veins at the venoatrial junction and an abnormally small left atrium.

Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and Meta-Regression.

OBJECTIVE: To quantify outcomes of infants (<1 year of age) diagnosed with pulmonary vein stenosis (PVS). STUDY DESIGN: MEDLINE (PubMed), Scopus, and Web of Science were searched through February 1, 2017, with no language restrictions. Publications including infants diagnosed with primary PVS, defined as the absence of preceding intervention(s), were considered. The study was performed according to Meta-analysis of Observational Studies in Epidemiology guidelines, the Systematic Reviews, and Meta-Analysis checklist, and registered prospectively. The quality of selected reports was critically examined. Data extraction was independently performed by multiple observers with outcomes agreed upon a priori. Data were pooled using an inverse variance heterogeneity model with incidence of mortality the primary outcome of interest. RESULTS: Forty-eight studies of 185 infants were included. Studies were highly diverse with regards to the participants, interventions, and outcomes reported. The median (range) age at diagnosis was 5.0 (0.1-11.6) months. Pooled mortality was 58.5% (95% CI 49.8%-67.0%, I2 = 21.4%). We observed greater mortality incidence among infants with 3 or 4 vein stenoses than in those with 1 or 2 vein stenoses (83.3% vs 36.1%; P < .01). We observed greater mortality among infants with bilateral than unilateral disease (78.7% vs 26.0%; P < .01). CONCLUSIONS: Studies of primary PVS during infancy are highly variable in their methodological quality and estimates of clinical outcomes; therefore, estimates of prognosis remain uncertain. Multicenter, interdisciplinary collaborations, including alignment of key outcome measurements, are needed to answer questions beyond the scope of available data.

Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis.

OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later. RESULTS: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious. CONCLUSION: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal.

[Pulmonary Vein Stenosis after Catheter Ablation of Atrial Fibrillation: clinical presentation as a Lung cancer. Case report.] / Estenosis de las venas Pulmonares post ablación de fibrilación auricular: presentación clínica similar a una neoplasia pulmonar. Reporte de caso.

Pulmonary vein stenosis is an infrequent complication of catheter ablation of atrial fibrillation. Its incidence varies widely from 0.3% to 6% according to different publications. The clinical presentation raises suspicion of more common pathologies. We present a case of pulmonary vein stenosis following catheter ablation of atrial fibrillation that resembled -clinically and on radiology- a lung neoplasm. We report a case of a 57-years-old male current smoker who developed pulmonary vein stenosis following catheter ablation of atrial fibrillation. The clinical (cough and hemoptysis), radiological (Positron-Emission Tomography positive pulmonary nodules) and bronchoscopy (edematous mucosa) were consistent with a lung neoplasm. Although previous cases of pulmonary vein stenosis resembling lung cancer have been published, we are unaware of previous description of pulmonary vein stenosis as cause of Positron-Emission Tomography positive pulmonary nodules. The case highlights the importance of considering pulmonary vein stenosis as a cause of cough, dyspnea and hemoptysis in patient with previous catheter ablation of atrial fibrillation in order to prevent unnecessary -often invasive- studies.

La estenosis de las venas pulmonares es una complicación infrecuente de la ablación por radiofrecuencia de la fibrilación auricular. Su incidencia oscila entre 0.3% a 6% de acuerdo a diferentes series. Sus manifestaciones clínicas son variadas y fácilmente confundibles con enfermedades pulmonares frecuentes, como neoplasias o infecciones. Presentamos el caso de un varón de 57 años con estenosis de venas pulmonares post ablación de una fibrilación auricular que se manifestó clínica (tos y hemoptisis), radiológica (nódulos pulmonares con captación aumentada en la tomografía por Emisión de Positrones) y endoscópicamente (mucosa congestiva y friable) en forma similar a una neoplasia de pulmón.Si bien casos de presentación similar a cáncer han sido descritos, no tenemos conocimiento de estenosis de venas pulmonares como causa de nódulos pulmonares hipercaptantes. El caso resalta la importancia de considerar la estenosis de venas pulmonares como causa de tos, disnea o hemoptisis en pacientes que hayan sido sometidos a una ablación por radiofrecuencia de arritmias auriculares.

Vena cava superior izquierda / Left superior vein cava

Mujer de 72 años con fibrilación auricular paroxística ingresó en la sala de electrofisiología para un aislamiento de venas pulmonares. La evaluación inicial con ecocardiografía intracardiaca reveló una dilatación significativa del seno coronario (fig. 1), la cual permitía el libre movimiento del catéter en su interior. De manera interesante, la ubicación del catéter decapolar en la parte lateral del seno generaba un aumento severo del automatismo atrial. Ante la sospecha de una vena cava superior izquierda persistente, se realizó reconstrucción tridimensional (EnSite NavX, St Jude Medical®) logrando visualizar el trayecto de la vena cava superior izquierda en su totalidad y su relación con el seno coronario (fig. 2). Durante el desarrollo embriológico normal, la vena cava superior izquierda involuciona y su trayecto intrapericárdico se convierte en la vena (ligamento) de Marshall. Aunque se ha documentado en aproximadamente el 0,2% de los pacientes llevados a estudio electrofisiológico (convirtiéndola en la alteración de las venas torácicas más común), pasa inadvertida debido a la ausencia de manifestaciones hemodinámicas1. De hecho, la mayoría se diagnostica de manera casual durante exámenes ordenados por otras razones.

Alterações eletrocardiográficas de um cão com estenose de valva pulmonar, antes e após terapia a base de maleato de enalapril e atenolol / Electrocardiographic alterations in a dog with pulmonary valve stenosis, before and after enalapril maleate and atenolol therapy

A estenose da valva pulmonar é uma malformação congênita cardíaca, caracterizada pelo comprometimento na via de saída do ventriculo direito levando a sinais clínicos de insuficiência cardíaca congestiva (ICC). Portanto, objetivou-se relatar as alterações eletrocardiográficas de um cão, fêmea, poodle, oito meses de idade, portador de estenose de valva pulmonar assintomático, antes e após terapia a base de maleato de enalapril e atenolol. Ao exame físico foi observado taquicardia e sopro sistólico em foco pulmonar grau V/VI e tricúspide grau III/VI, com o diagnóstico firmado por meio dos exames radiográfico, eletrocardiográfico e ecodopplercardiográfico; e terapêutica instituída a base de maleato de enalapril (0,50mg/kg) uma vez ao dia e atenolol (0,25 mg/kg) a cada 12 horas, ambos por via oral. Inicialmente, o eletrocardiograma (ECG) apontou taquicardia sinusal (250 bpm) e aumento na amplitude das ondas PmV, SmV e TmV, sugerindo sobrecarga atrial e ventricular direita, hipóxia do miocárdio, com desvio eixo cardíaco à direita. Com 15, 90, 120 e 210 dias de terapia subsequentes, os ECGs demonstraram ritmo sinusal com redução gradativa da frequência cardíaca e nos valores de amplitude das ondas P, S e TmV. As alterações descritas sugeriram a melhora do traçado eletrocardiográfico em virtude da redução na pré e pós-carga cardíaca, obtida por meio da terapia. Portanto, concluiu-se que os eletrocardiogramas realizados durante o período de sete meses subseqüentemente, evidenciaram alterações eletrocardiográficas que demonstram os beneficios da terapia baseada no maleato de enalapril associado ao atenolol no cão portador da estenose da válvula pulmonar.

The pulmonary valve stenosis is a congenital heart malformation characterized by impairment in the outflow of the right ventricle leading to clinical signs of congestive heart failure (CHF). Therefore, the objective was to report the electrocardiogram (ECG) changes of a female 8 months old dog, poodle, with asymptomatic stenosis of the pulmonary valve before and after therapy the basis of enalapril and atenolol. Physical examination was observed tachycardia and pulmonary systolic murmur grade V / VI and tricuspid grade III / VI, with the diagnosis confirmed through radiographic, ECG and echocardiographic examinations, and therapy based on enalapril maleate (0.50 mg/kg) once daily and atenolol (0.25 mg/kg) every 12 hours, both orally. Initially, the ECG showed sinus tachycardia (250 bpm) and an increase in amplitude of the waves PmV, SmV and TmV, suggesting atrial and right ventricular myocardial hypoxia, cardiac axis deviation to the right. At 15, 90, 120 and 210 days of therapy subsequent ECGs showed sinus rhythm with a gradual reduction in heart rate and amplitude values of P waves, S and TmV. The changes described suggest improvement of the electrocardiographic tracing due to the reduction in pre-and post-cardiac load, obtained through therapy. Therefore, we concluded that the ECG performed during the seven months subsequently showed electrocardiographic changes that demonstrate the benefits of therapy based on enalapril maleate associated with atenolol in the dog with pulmonary valve stenosis.