Congenital Coronary Arteriovenous Fistula Presenting with Syncope

Previous reports of syncope in patients with coronary arteriovenous fistula (CAVF) have theorized that it occurs secondary to a coronary steal phenomenon. We present a case of syncope in a young woman with a CAVF and no anatomic substrate for coronary steal.     

Neonatal Pulmonary Artery Thrombosis Presenting as Persistent Pulmonary Hypertension of the Newborn

Pulmonary artery thrombosis in neonates occurs rarely. This report describes the case of a term infant with a pulmonary artery thrombosis presenting as persistent pulmonary hypertension of the newborn. The risk factors identified in the case included maternal diabetes and heterozygous factor V Leiden deficiency. The pulmonary thrombus was successfully treated with percutaneous catheter-based embolectomy.     

Congenital Chylothorax and Persistent Pulmonary Hypertension of the Neonate

ABSTRACT. In this case of congenital chylothorax, an associated persistent pulmonary hypertension was a severe complication which has not hitherto been described. Recurrence of the chylothorax with the introduction of regular formula is also reported.     

Congenital Arteriovenous Malformation Between the Subclavian Artery and Superior Vena Cava Presenting in Neonatal Heart Failure

Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure. This unusual fistulous connection has only rarely been reported in the literature, and in those reports, it has not resulted in neonatal heart failure.     

ANCA-associated Vasculitis Presenting as Severe Pulmonary Hypertension and Right Heart Failure

Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor.     

Diffuse Pulmonary Arteriovenous Malformation in a Child with Polysplenia Syndrome

Pulmonary arteriovenous malformation (PAVM) is usually seen as a well-circumscribed cysticmass. In this communication we describe a diffuse arteriovenous malformation in a 5-month-old infant. The lesion was seen only at the microscopic level and was associated with abdominal heterotaxy, atrial situs solitus, polysplenia, interrupted inferior vena cava, atrial septal defect, and atrioventricular canal. This uncommon association may be within the spectrum of polysplenia syndrome.     

Coil Embolization of a Neonatal Pulmonary Arteriovenous Malformation

Pulmonary arteriovenous malformation (PAVM), as a part of Osler-Weber-Rendu Syndrome, in the neonate, is a rare hereditary vascular malformation. Large intrapulmonary right-to-left shunting, causing hypoxaemia and cyanosis, can be a life-threatening condition. Repeated transcatheter coil embolization procedures proved to be a favorable strategy to improve systemic arterial oxygen saturation, with a good outcome in a newborn child. While the radiation dose was high, the use of this amount of radiation was felt to be justified and its effects considered tolerable in the treatment of this patient's serious malformation.     

新生儿持续肺动脉高压

新生儿持续肺动脉高压并不是一种单一的疾病,而是由多种因素所导致的临床综合征.不同病因及不同病理类型的患儿对临床治疗的反应有很大的差异.除传统的药物降低肺动脉压力外,机械通气、一氧化氮吸入等新的方法 均逐渐成为安全有效的治疗手段.     

Congenital Misalignment of Pulmonary Veins with Alveolar Capillary Dysplasia Causing Persistent Neonatal Pulmonary Hypertension: Report of Two Affected Siblings

Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia. Received February 2, 1999; accepted June 28, 1999.     

Idiopathic Severe Pulmonary Hypertension in an Infant with Pulmonary Infection

We report a 5-month-old infant who showed typical echocardiographic findings of primary pulmonary hypertension without the typical histopathological findings and who recovered from severe pulmonary hypertension. Histopathological findings revealed mild thickening of small pulmonary arteries and activated macrophages in the lung. Some cases with idiopathic severe pulmonary hypertension in infants are associated with pulmonary infection.     

Adrenoleukodystrophy Associated with Cerebral Arteriovenous Malformation

Adrenoleukodystrophy (ALD) is now considered to be an X-linked recessive peroxisomal disorder. We report the case of a patient with ALD associated with a left parietal arteriovenous malformation (AVM) who deteriorated progressively six months after recovery from a hemorrhage of the AVM. Serial CT scans and MR imagings showed progressive demyelination spreading from the affected area. This case suggests that local brain damage due to AVM hemorrhage may accelerate the progress of demyelination.     

Congenital Bilateral Renal Arteriovenous Malformation: An Unrecognized Cause of Renal Failure

Congenital renal arteriovenous malformations (AVM) are very rare abnormal communications between arteries and veins. These lesions are almost always unilateral, predominantly in the right kidney, and usually asymptomatic until adulthood. We present a unique case of bilateral renal AVM in a 10-year-old white boy who developed renal failure requiring renal transplantation. Microscopic sections of an atrophic right and a slightly larger left kidney with tortuous and dilated hilar vessels showed elaborate derangement of arteries and veins insinuating between lobules. Glomeruli were diffusely enlarged with increased number of capillary loops. Glomerular basement membrane reduplication and fibrinoid necrosis was focally noted. Electron microscopy demonstrated absence of electron-dense deposits or mesangial interposition excluding membranoproliferative glomerulonephritis. We believe that the glomerular lesions are secondary to congenital renal arteriovenous malformation. To our knowledge, bilateral arteriovenous malformation in infancy is not previously described.     

Congenital Cystic Adenomatoid Malformation Within An Extralobar Pulmonary Sequestration

A type II congenital cystic adenomatoid malformation (CCAM II) occurring within an extralobar pulmonary sequestration (ELS) is described. This large malformation and accompanying pulmonary hypoplasia were diagnosed prenatally by ultrasound examination at 24—25 weeks gestational age. Based upon this diagnosis, the pregnancy was terminated. Autopsy revealed a 40.5 g supradiaphragmatic sequestration, severely hypoplastic lungs, and a hypoplastic heart. Morphologic and clinical features of both CCAM and ELS are compared. CCAM occurring within an ELS has been reported on at least twelve occasions. All the malformations in which the type was reported were of the less differentiated types II or III. We offer an explanation of this fact based on the timing of the embryologic maldevelopment.     

可调式房间隔内漏术在先天性心脏病并重度肺动脉高压术中的应用

目的探讨可调式房间隔内漏术在先天性心脏病（CHD）并重度肺动脉高压（PH）术中的方法和应用价值。方法选择27例重度CHD患儿。男19例，女8例；年龄4～14岁；体质量13．7～42．0kg。其中房间隔缺损（ASD）11例，室间隔缺损（VSD）10例，动脉导管未闭（PDA）4例，Ebstein畸形伴重度三尖瓣关闭不全2例。术前经胸片、心电图、超声心动图、心导管和心血管造影检查，诊断为有双向分流的重度PH的CHD，常规手术属于禁忌证。结果经可调式房间隔造瘘和畸形矫正处理，14例手术结束关胸时闭合造瘘口，7例术后第2天、3例第3天闭合造瘘口，1例第4天闭合，但术后并脓胸，感染控制后出院，1例VSD造瘘口永久开放，1例PDA术后死于低心排。手术总有效率92．6％，接近常规手术效果和风险。结论可调式房间隔造瘘术简便易行，能有效控制术后PH风险，为多数近终末期分流型CHD创造手术机会。     

Congenital Alveolar Capillary Dysplasia: A Developmental Vascular Anomaly Causing Persistent Pulmonary Hypertension of the Newborn

The clinical course and histologic findings are presented of an infant with an unusual form of pulmonary dysplasia. Characteristic sonographic findings and progressive hypoxemia led to the diagnosis of persistence of the fetal circulation. The patient expired despite ventilatory and pharmacologic intervention. Postmortem findings of severe pulmonary capillary hypoplasia, despite normal anatomical and biochemical parenchymal maturation, were observed. It is suggested that factors controlling pulmonary capillary maturation may be significantly different from those involved in airway and pulmonary parenchymal development.     

Pulmonary Arteriovenous Malformation in Patients with Total Cavopulmonary Shunt: What Role Does Lack of Hepatic Venous Blood Flow to the Lungs Play?

We examined the etiology of a long-term postoperative complication, pulmonary arteriovenous malformation (PAVM), in a patient who underwent total cavopulmonary shunt (TCPS). The patient underwent TCPS at the age of 3 years and 1 month, and a PAVM developed. At the age of 10 years, TCPS was switched to total cavopulmonary connection to facilitate circulation of hepatic venous blood to the pulmonary artery. As a result, oxygen saturation increased. These findings suggest that the absence of pulmonary circulation of hepatic venous blood is involved in the development of PAVM.     

Transcatheter Occlusion of a Large Pulmonary Arteriovenous Malformation Using the Amplatzer Vascular Plug

Pulmonary arteriovenous malformation (AVM) is a rare entity with well-described signs, symptoms, and complications. Pulmonary AVMs can be congenital or acquired. They have been described in the setting of severe liver disease and after palliation with a Glenn shunt in which the hepatic venous blood flow has been excluded from the pulmonary blood flow. A variety of surgical and transcatheter interventions have been used to occlude AVMs. We report the use of the Amplatzer vascular plug to successfully occlude a pulmonary AVM in a 12-year-old patient.     

肺动脉内置管应用前列腺素E1及单向活瓣补片治疗先天性心脏病并严重肺动脉高压

目的 探讨先天性心脏间隔缺损并重度肺动脉高压(PH)患儿的外科治疗方法及疗效.方法 选择本院2003年7月-2007年6月23例先天性心脏病并重度PH病例,全部采用单向活瓣补片进行心脏间隔缺损修补,术中留置肺动脉内置管泵入前列腺素E1(PGE1),并二尖瓣明显返流者同期行二尖瓣成形术,尖瓣返流者行Devega缩环成形术.术后采用超声心动图监测分流情况,并比较患儿术前、术后肺动脉压(PAP)及血气分析指标变化.采用SPSS 10.0软件进行统计学分析.结果 患儿术后平均系统血压与术前比较无显著性差异,而PAP较术前明显下降,血气指标较术前有明显改善;肺动脉内持续应用PGE1降低PAP效果显著;随访发现1例术后持续PH存在,单向活瓣持续右向左分流,余22例分别于手术后第3～6个月活瓣关闭、分流消失.结论 单向活瓣补片修补缺损、肺动脉内置管泵入PGE1能明显降低PAP,改善血气指标,增加围手术期安全性,同期行必要的瓣膜成形术有利于降低PAP,心脏间隔缺损并重度PH应严格把握手术适应证.     

Prostacyclin Treatment for Persistent Pulmonary Hypertension of the Newborn

To study the effect of prostacyclin treatment on pulmonary arterial pressure (PAP), systolic pressure (BP), and systemic oxygenation, eight infants with persistent pulmonary hypertension of the newborn (PPHN) born between 34 and 42 weeks' gestation and having a birth weight of 2540–4130 g were studied using Doppler echocardiography. At a mean age of 19 hours (range 3–32 hours), despite maximal ventilator therapy and an FiO₂ of 1.0, the mean PaO₂/PAO₂ was 0.07 (range 0.04–0.09) and the AaDO₂ was 616 mmHg (range 521–654 mmHg). After volume correction and during inotropic medication with dopamine and dobutamine, the mean PAP by echocardiography was 68.6 ± 6.5 mmHg and the mean BP 59.8 ± 4.8 mmHg. Prostacyclin infusion was then started at a dose of 20 ng/kg/min and increased stepwise to a mean dose of 60 ng/kg/min (range 30–120 ng/kg/min) over 4–12 hours, at which time PAP decreased to 49.2 ± 3.5 mmHg (p= 0.0005) and BP to 53.2 ± 9.1 mmHg (p= 0.17); the PAP thereafter remained below the BP. After 72 hours of prostacyclin infusion, PAP was 49.6 ± 18 mmHg, BP 66.1 ± 5.4 mmHg, PaO₂/PAO₂ 0.14 ± 0.12, and AaDO₂ 428 ± 189 mmHg at FiO₂ 0.65. The median duration of prostacyclin infusion was 3.6 days and of respirator treatment 7.0 days. All patients survived without extracorporeal membrane oxygenation. At 6–12 months, none of the patients had severe central nervous system complications, but two had bronchopulmonary dysplasia. These findings indicate that prostacyclin is able to reverse the right-to-left shunt in PPHN by decreasing PAP, and that systemic hypotension can be prevented with adequate volume correction and inotropic medication.