The Use of Low-Dose Recombinant Tissue Plasminogen Activator to Treat a Preterm Infant with an Intrauterine Spontaneous Arterial Thromboembolism.

Neonatal thromboembolic events are rare, and only a few cases of intrauterine spontaneous arterial thromboembolisms have been reported in the literature. Thrombolytic therapy with recombinant tissue plasminogen activator is usually the preferred treatment because it has a short half-life, fewer systemic side effects, and a strong, specific affinity for fibrin. Protocols vary from center to center, but there is still no consensus regarding the proper dosage or treatment duration. Herein, we present the case of an intrauterine spontaneous arterial thromboembolism in a preterm infant that completely resolved after being treated with low-dose recombinant tissue plasminogen activator (0.02 mg/kg/h).

Simultaneous stenting of tightly stenosed patent ductus arteriosus and pulmonary artery bifurcation using two stents (Y stenting): an innovative technique.

Stenting of patent ductus arteriosus (PDA) is a palliative technique that is evolving as an alternative to shunt surgery. Patients with duct-dependant pulmonary circulation and branch pulmonary artery stenosis are often palliated by shunt surgery with repair of branch pulmonary arteries under cardiopulmonary bypass. We present here an 8-month-old male child with duct-dependant pulmonary circulation with bifurcation stenosis who was palliated successfully by transcatheter means. He had stenosed PDA with tight pulmonary artery bifurcation stenosis and underwent successful "Y" stenting of PDA with simultaneous deployment of two stents. He successfully underwent bidirectional Glenn surgery 8 months after the procedure. Simultaneous stenting of bifurcation stenosis of branch pulmonary arteries with two stents has not been described in the literature.

Popliteal artery entrapment syndrome with thrombosed popliteal aneurysm: multidetector computed tomography angiography findings of a case.

Popliteal artery entrapment syndrome (PAES) is a rare congenital vascular pathology caused by the compression of the popliteal artery by adjacent muscle and tendinous structures. Popliteal artery aneurysm associated with this syndrome is extremely rare. A 45-year-old male suffering from pain at the right lower extremity during exercise was admitted to our hospital. Physical examination and Doppler imaging revealed a weak pulse at the posterior tibial artery and no pulse at dorsalis pedis and anterior tibial arteries. The patient was further evaluated with multidetector computed tomography angiography (MDCTA). MDCTA revealed PAES due to compression of the accessory fibers of the gastrocnemius muscle and related thrombosed popliteal aneurysm.

Role of CT angiography in the diagnosis and treatment of popliteal vascular entrapment syndrome.

OBJECTIVE: Popliteal vascular entrapment syndrome is an uncommon congenital abnormality of the anatomic relations between the popliteal vessels and the neighboring musculotendinous structures. The purpose of this essay is to describe the CT angiographic findings in the diagnosis and treatment of this syndrome. CONCLUSION: Digital subtraction angiography is of limited value in the evaluation of popliteal vascular entrapment syndrome and has been replaced by noninvasive imaging techniques, such as Doppler sonography, CT angiography, MRI, and MR angiography.

Myocardial ischemia secondary to congenital hypoplastic left coronary cusp in adult.

Coronary arterial obstruction associated with congenital aortic valve disease is rare in childhood, and has not been reported in adult. Here we reported a 49-year-old healthy woman with hypoplastic left coronary cusp resulting in myocardial ischemia in the territory of left main coronary artery.

Tetralogy of fallot: a possible etiology for narrowing of the right pulmonary artery.

RATIONALE AND OBJECTIVES: Patients with tetralogy of Fallot have right ventricular outflow and pulmonary valve stenosis. They may also have more distal peripheral pulmonary artery stenoses. Peripheral stenosis is believed to be part of the spectrum of the genetic manifestation of tetralogy. Our hypothesis was that narrowing of the right pulmonary artery may be due to compression from the adjacent dilated ascending aorta. MATERIALS AND METHODS: We identified 27 patients with tetralogy of Fallot who underwent magnetic resonance imaging scanning postoperatively, most often for evaluation of pulmonary valve stenosis and regurgitation. On the axial image at the level of the right pulmonary artery, we measured the transverse diameters of the ascending and descending aorta, and the minimum and maximum right pulmonary artery diameters. RESULTS: There was a significant correlation between the ratios of the ascending:descending aorta diameters and the maximum:minimum right pulmonary artery diameters. Thus, increasing ascending aorta size is associated with decreasing size of the adjacent right pulmonary artery. CONCLUSIONS: In our patient population with tetralogy of Fallot, right pulmonary artery narrowing is at least partly because of compression from the adjacent enlarged ascending aorta.

CT angiography and MRI in patients with popliteal artery entrapment syndrome.

OBJECTIVE: Popliteal artery entrapment syndrome (PAES) is an uncommon congenital anomaly affecting young adults who present with symptoms of calf claudication. It is characterized by various anomalous anatomic relationships between the muscle and arteries in the popliteal fossa, resulting in extrinsic arterial compression. The purpose of this study was to evaluate the potential role of CT angiography (CTA) and MRI in the diagnosis of PAES. MATERIALS AND METHODS: Eight patients (11 legs) who underwent surgical treatment for PAES over a 4-year period were included in this study. All of the cases showed various anomalous relationships between the popliteal artery and the neighboring muscular structures. CTA and MRI showed the detailed anatomy of the region well enough to reveal the cause of arterial entrapment. CTA defined the location and length of the occluded segment and collateral circulation. RESULTS: Characterization and classification based on CTA and MRI findings were consistent with intraoperative photography. CONCLUSION: CTA and MRI can show anatomic variations in the popliteal fossa and may be valuable in the diagnosis of PAES in young adults presenting with intermittent claudication.

Diagnosis of congenital obstructive aortic arch anomalies in Chinese children by contrast-enhanced magnetic resonance angiography.

OBJECTIVE: The purpose of this study was to evaluate the accuracy of contrast-enhanced magnetic resonance angiography for the diagnosis of congenital obstructive aortic arch anomalies in children and compare it with transthoracic echocardiography and other MR imaging techniques (ECG gated T1-weighted spin-echo imaging and gradient-echo cine imaging). MATERIALS AND METHODS: Contrast-enhanced magnetic resonance angiography, ECG gated T1-weighted spin-echo imaging, and gradient-echo cine imaging were performed for the diagnosis of congenital obstructive aortic arch anomalies in 416 patients from April 1999 to March 2005 (age range, 3 days to 12 years; mean age, 2.4 years) using a GE 1.5T MR scanner. Transthoracic echocardiography was performed in all patients prior to MR examination. Surgery and/or conventional X-ray angiocardiography were done in all patients to determine the final diagnosis. RESULTS: The population consisted of 416 patients. Congenital obstructive aortic arch anomalies were diagnosed in 213 patients and ruled out in 203 patients by operation and/or conventional X-ray angiocardiography. Among the 213 patients with anomalies, coarctation of aorta was diagnosed in 174, interruption of aortic arch was diagnosed in 35, and persistent fifth aortic arch with fourth aortic arch interruption was diagnosed in 4 patients. Among the 35 patients with interruption of aortic arch, 21 were of type A, and 14 were of type B. The diagnostic sensitivity, specificity and accuracy of contrast-enhanced magnetic resonance angiography for congenital obstructive aortic arch anomalies were 98% (208/213), 99% (201/203) and 98% (409/416), respectively. The diagnostic sensitivity, specificity and accuracy of transthoracic echocardiography were 88% (187/213), 92% (186/203) and 90% (373/416), respectively. The diagnostic sensitivity, specificity and accuracy of other MR imaging techniques (ECG gated T1-weighted spin-echo imaging and gradient-echo cine imaging) were 89% (189/213), 84% (170/203) and 86% (359/416), respectively. CONCLUSION: Contrast-enhanced magnetic resonance angiography is a reliable, noninvasive imaging technique for the diagnosis of congenital obstructive aortic arch anomalies in children. Occasionally, even more information can be obtained from this technique than from conventional X-ray angiocardiography. Contrast-enhanced magnetic resonance angiography is superior to transthoracic echocardiography and other MR imaging techniques (ECG gated T1-weighted spin-echo imaging and gradient-echo cine imaging) for diagnosis of congenital obstructive aortic arch anomalies in children.

Relationship of the celiac trunk with median arcuate ligament of the diaphragm

RESUMEN: En el presente trabajo se investigaron las posiciones del ligamento arqueado mediano con relación al tronco celíaco, y las medidas de sobreposición y distancia entre estas dos estructuras. Además, fueron realizadas medidas de algunas variables como la longitud y el diámetro del tronco celíaco, estudios histológicos de los pilares del diafragma y de su ligamento arqueado mediano. Utilizamos 63 cadáveres fijados en solución de formalina al 10% y 20 cadáveres no fijados, adultos, de ambos sexos. Las disecciones del área del tronco celíaco fueron realizadas después de disecar la cavidad peritoneal, en los laboratorios de la Disciplinas de Anatomía de la UNIFESP-EPM, UNILUS y UNISA, y durante las necropsias en los Servicios de Verificación de Óbitos de la UNIFESP-EPM y USP, Brasil. Para el análisis morfológico, a nivel de microscopía de luz, de tres cadáveres no fijados escogidos al azar, fueron retirados fragmentos del ligamento arqueado mediano de 0.5 cm de alto por 1.5cm de largo. Luego fueron procesados según técnicas tradicionales de inclusión. Se efectuaron cortes de 5 µm, los cuales fueron teñidos con hematoxilina-eosina y tricrómico de Masson. Los resultados obtenidos permitieron llegar a las siguientes conclusiones: 1) Las posiciones del ligamento arqueado mediano en el tronco celíaco fueron independientes del sexo. En 12 (14.46%) de 83 cadáveres, se pudo observar al tronco celíaco distante del ligamento arqueado. En 35 (42.17%) este ligamento era tangente al tronco celíaco y en 36 (43.37%) se sobreponía a este vaso. El promedio de la distancia entre el ligamento arqueado mediano y el tronco celíaco fue de 0.94 cm y la de sobreposición 0.42 cm; 2) los resultados histológicos del análisis del ligamento arqueado mediano mostraron al tejido conjuntivo denso, infiltrado por células adiposas, vasos sanguíneos y nervios. Fueron observadas fibras musculares estriadas, entremezcladas con fibras colágenas.

Avaliação imunohistoquímica das células inflamatórias presentes na parede de artérias pulmonares periféricas de pacientes com doença vaso-oclusiva pulmonar secundária a defeitos congênitos / Immunohistochemical evaluation of inflammatory cells in the walls of peripheral pulmonary arteries from patients with pulmonary vasoclusive disease secondary to cardiac congenital defects

Para avaliar a hipótese da presença de inflamação em artérias pulmonares periféricas de pacientes com hipertensão pulmonar (HP) decorrente de cardiopatias congênitas, foram quantificadas células inflamatórias através de marcação imunohistoquímica em biópsias de 26 pacientes e comparadas com 11 controles sem cardiopatia. Detectou-se quantidades semelhantes de células inflamatórias nos dois grupos, mas com predomínio de linfócitos T no grupo controle e de macrófagos jovens no grupo HP. Esses achados podem estar relacionados com a redução do estímulo dependente de macrófagos para diferenciação e maturação de linfócitos T nos cardiopatas e/ou a deficiência imunológica primária nesses pacientes./To evaluate the hypothesis of increased inflammation in peripheral pulmonary arteries from patients with pulmonary hypertension secondary to congenital cardiac shunts, we quantified the inflammatory cells with the aid of immunohystochemistry in 26 biopsies (HP group), comparing them to 11 patients with no cardiac disease. Similar quantities of inflammatory cells were observed in the two groups, with a predominance of T-lymphocytes in the controls and of young macrophages in the HP group. These findings could be related to a reduction of macrophagic stimulus to the differentiation and maturation of T-lymphocytes and/or to a primary immunological deficiency in patients...

The efficacy and safety of stent redilatation in congenital heart disease.

OBJECTIVES: To assess the extent of neointimal proliferation and the safety and efficacy of stent redilatation in patients with stents implanted for aortic coarctation or branch pulmonary artery stenosis. DESIGN: Retrospective observational study. SETTING: Tertiary referral centre. PATIENTS AND INTERVENTIONS: Of 60 patients with Palmaz stents, 12 with coarctation and 12 with branch pulmonary artery stenosis (with 21 stented sites) underwent recatheterisation and stent redilatation. RESULTS: Neointimal thickening > 1 mm was detected in six of the 12 coarctation stents and at nine of the 21 stented sites in branch pulmonary arteries (five of which had been overdilated at implantation). Eight of the coarctation stents were electively underdilated at implantation. Coarctation stent redilatation increased median (range) lumen diameter from 9.2 (6.3-11.1) mm to 11.7 (9.8-14.1) mm (p = 0.002), decreased gradient from 10.5 (0-20) mm Hg to 0.5 (0-15) mm Hg (p = 0.008), and increased the ratio of stent diameter to descending aorta diameter from 0.66 (0.38-1.02) to 0.85 (0.52-1.2) (p = 0.008). Pulmonary artery stent redilatation increased lumen diameter from 6.9 (3.8-13.5) mm to 8.8 (4.8-14.1) mm (p < 0.001), decreased gradient from 24 (2-62) mm Hg to 12 (0-29) mm Hg (p < 0.001), and increased the ratio of stent diameter to diameter of distal pulmonary artery from 0.66 (0.44-1.5) to 0.86 (0.48-1.88) (p = 0.001). Dilatation of one peripheral pulmonary artery stent resulted in rupture of the vessel distal to the stent. CONCLUSIONS: Neointimal proliferation is precipitated by overdilating stents at implantation. Redilatation using balloons matched to distal vessel diameter increases stent lumen size, but may not optimise vessel diameter. Redilatation is effective whether the indication for redilatation is a resistant stenosis at implantation, underexpansion at implantation, neointimal proliferation, or relative stenosis caused by growth.

Bulboventricular foramen resection: hemodynamic and electrophysiologic results.

BACKGROUND: The two major surgical approaches to the relief of bulboventricular foramen (BVF) obstruction in patients with single left ventricle (LV) are the Damus-Kaye-Stansel (DKS) procedure or direct BVF resection. Theoretical advantages of the DKS include better out-flow gradient relief, lower potential incidences of postoperative heart block and lower incidences of reoperation. Potential disadvantages of this approach include increased semilunar valvar insufficiency, lack of feasibility when attempting septation-type operations for univentricular hearts, and a technically more difficult operation. We report the results of direct surgical BVF resection. METHODS: From June 1990 to June 1999, 9 patients had direct BVF resection performed at our institution. The median age at surgery was 16.5 years (range 1 month to 27 years). Diagnoses in these patients were [S,L,L] single LV (n = 8) and [S,D,D] single LV tricuspid atresia (n = 1). Eight of 9 patients had pulmonary artery bands placed either before BVF resection or at the same time as this procedure. Three patients required reoperation for reobstruction at the BVF (12 total operations in 9 patients). RESULTS: Median preoperative peak systolic gradient across the BVF measured at cardiac catheterization was 47 mm Hg (range 10 to 63 mm Hg). The median peak gradient measured by Doppler echocardiography was 44 mm Hg (range 5 to 125 mm Hg). Eight of 9 patients survived the operation to discharge from the hospital and 7 of 9 are alive at follow-up. At a median follow-up of 22 months (range 5 to 76 months), 8 of 8 surviving patients had an unobstructed BVF as determined by qualitative two-dimensional echocardiography and Doppler color flow imaging. There was one perioperative and one late death 5 months postoperatively (secondary to fungal sepsis). No patient developed new or worsened aortic insufficiency after BVF resection. Eight of 9 patients had no change in AV nodal conduction after surgery. One patient developed Mobitz II heart block requiring postoperative implantation of a pacemaker. CONCLUSIONS: Direct resection of an obstructive BVF can be performed with total relief of obstruction although reoperation may be required. Atrioventricular nodal function can be preserved in most patients with this operative approach, including those with [S,L,L] segmental anatomy.

Comparison of three-dimensional contrast-enhanced magnetic resonance angiography and axial radiographic angiography for diagnosing congenital stenoses in small pulmonary arteries.

Accuracy of 3-dimensional contrast-enhanced magnetic resonance angiography (MRA) in diagnosing morphology of the branch pulmonary artery (PA) was evaluated in 73 patients (aged 7.2 +/- 6.4 years [mean +/- SD]) with various congenital heart diseases. The presence or absence of localized stenosis of branch PAs, PA diameter, and Nakata's PA index were determined on MRA and axial radiographic angiography, and the results were compared. Sensitivity, specificity, and overall accuracy in detecting branch PA stenoses were 92.7%, 96.2%, and 95.2%, respectively. Correlations between axial radiographic angiography and MRA were excellent in measuring PA diameter (r = 0.956, SEE = 1.49 mm, n = 139) as well as PA index (r = 0.839, SEE = 48.9, n = 37); both p < 0.0001. Bland-Altman plots showed a mean difference +/- SD for PA diameter of 0.17 +/- 1.51 mm and for PA index of 8.5 +/- 50.1. When the main right and left PAs were taken as the first generation, the most distal branches visible on MRA were the 4.7 +/- 0.7 generation with breath-holding (n = 23) and the 3.7 +/- 0.5 without breath-holding (n = 50), respectively (p < 0.0001). Both intra- and interobserver variabilities of MRA measurements were few (9.5 +/- 11.6% and 13.5 +/- 15.0%, respectively, n = 139). In conclusion, 3-dimensional contrast-enhanced MRA enables us to document branch PA morphology clearly in infants and adult patients with congenital cardiovascular defects.

Immediate post-operative balloon dilatation of neonatal long segment stenosis of the descending thoracic aorta.

During surgical repair of a neonatal coarctation it was recognized that this was a rare, previously undescribed form of stenosis of the entire descending thoracic aorta. A few hours after the end-to-end surgical repair, the child underwent successful balloon angioplasty, involving the entire descending thoracic aorta, and in which we intentionally avoided the surgical site.

MR imaging of non-visualized pulmonary arteries at angiography in patients with congenital heart disease.

The aim of this study was to evaluate whether MR could depict pulmonary arterial anatomy in more detail than routine angiography in patients with congenital interruption or acquired occlusion of the left pulmonary artery or pulmonary atresia. This study included 10 patients with tetralogy of Fallot (n=6) or pulmonary atresia with ventricular septal defect (n=3) or aorticopulmonary window (n=1) diagnosed by cardiac angiography and MR. Surgical confirmation was made in seven patients. Interruption of the proximal left pulmonary artery, diagnosed at the time of evaluation, was found in seven patients and acquired obstruction of the hilar pulmonary artery (PA) was found in two at cardiac angiography. In the remaining one patient with pulmonary atresia and an occluded palliative shunt, the central PA was not visualized at angiography. MR showed 3-6 mm-sized hilar PAs in five and a central PA in a patient with pulmonary atresia. In 4 of 6 (67%) surgically-proven patients with congenital or acquired left PA obstruction, the status of the PA distal to the obstruction was correctly diagnosed with MR. In conclusion, MR is an effective modality in depicting sizable PAs when routine angiography fails to visualize the PA anatomy.