Isolated neonatal ductus arteriosus aneurysm.

OBJECTIVE: A prospective study was performed to evaluate the incidence, clinical manifestations and outcome of ductus arteriosus aneurysm (DAA) in full-term neonates. BACKGROUND: Ductus arteriosus aneurysm has been considered to be a rare congenital lesion and a potentially fatal abnormality. METHODS: A total of 548 full-term neonates received echocardiographic screening. RESULTS: There were 48 (8.8%) patients (28 boys and 20 girls) with DAA detected by echocardiography. The maximal diameter of the DAA ranged from 6.5 to 11.2 mm (8.2 +/- 1.2 mm). All cases were asymptomatic. There were no significant differences in gender, gestational age, maternal age or Apgar score between the newborns with or without DAA. Newborns with DAA had a higher birth body weight, higher incidence of maternal gestational diabetes mellitus and more mothers with blood group A, compared with newborns without DAA (p < 0.05). Follow-up echocardiograms showed spontaneous closure of the ductus arteriosus in all patients except those without DAA. The DAA became progressively smaller after ductal closure in 33 patients (70.2%) and completely disappeared by 7 to 35 days of life. The other 14 patients (29.8%) with DAA had echocardiographic evidence of progressive formation of thrombi between the third and tenth day of life. The DAA and thrombi spontaneously disappeared in all patients by one month after birth. CONCLUSIONS: There is a higher incidence of DAA with a good outcome in our series compared with previous reports. We speculate that the presence of DAA may be a normal variant of the ductal bump and part of a normal process of spontaneous ductal closure in full-term neonates.     

Neonatal Systemic Thromboembolism Secondary to Ductus Arteriosus Aneurysm and Patent Foramen Ovale

Ductus arteriosus aneurysm (DAA) can be associated with neonatal thromboembolism. We report a neonate with systemic thromboembolism causing acute cerebral infarction with DAA, arterial, and venous thrombosis, discovered on CT angiography. The role of DAA was suspected as a potential etiology of systemic thromboembolism in this case. CT angiography with three‐dimensional reconstruction was valuable delineating the adjunctive vascular structures. Screening for presence of DAA may be considered in the neonatal thromboembolism.     

Ductus arteriosus rupture as a balloon catheter atrioseptostomy complication

A newborn with transposition of the great arteries presented with rupture of the ductus arteriosus after balloon catheter atrioseptostomy. The necropsy study demonstrated persistent ductus patency, and a 0.5-cm-long horizontal fissure could be observed. On microscopy, there was laceration of the intimal layer, with wall dissection and focal hemorrhage extending to the adventitia. Ductus rupture was attributed to the wall weakess, as a consequence of prostaglandin E1 administration.     

Ductal Arteriosus Aneurysm,Right Aortic Arch,and Isolated Left Subclavian Artery in a Neonate

Ductal arteriosus aneurysm (DAA) is a well‐recognized condition, especially in infancy, and is usually asymptomatic. We report the first case of a newborn who presented with significant inspiratory stridor and, using multiple imaging investigations, was subsequently diagnosed with the rare constellation of a congenital DAA, a right aortic arch and an isolated left subclavian artery with normal intra‐cardiac anatomy. The patient underwent surgical resection of the DAA with significant improvement in symptoms.     

Formation of intimal cushions in the ductus arteriosus as a model for vascular intimal thickening. An immunohistochemical study of changes in extracellular matrix components.

There is a great resemblance in the sequence of events that take place in the pathological development of intimal thickening, so called arteriosclerosis and the formation of intimal cushions in both the normal ductus arteriosus (DA) and the persistent ductus arteriosus (PDA). The human DA was used as a model to study the changes in the extracellular matrix during this process with immunohistochemistry. The formation of intimal cushions was studied in 4 normal fetal DA, 4 normal mature DA and 3 persistent DA. The process of intimal thickening in the fetus starts in the second trimester of pregnancy with an accumulation of glycosaminoglycans in the subendothelial region (SER), accompanied by separation of endothelial cells from the internal elastic lamina and followed by migration of smooth muscle cells into the subendothelial region. This phenomenon was also observed in the mature DA in the neonate, indicating that cushion formation is a continuous process. Intimal cushions had also developed in the persistent DA, although they were morphologically different from the cushions found in the normal mature DA. It was remarkable that two elastic lamellae could be distinguished: one at the original site on the borderline of intimal cushion and media and the other in a subendothelial position. The endothelial cells were firmly attached to this subendothelial lamina, which was wrapped in the basal lamina components laminin and type IV collagen. The main morphological difference between the normal mature DA and the persistent DA is the close relation between endothelial cells and the subendothelial elastic lamina, suggesting an altered elastin metabolism in the PDA. PGI2 synthase was increased in the wall of both the normal and persistent DA as compared with the aorta. It may be related to a role of PGI2 in the formation of intimal cushions.     

彩色多普勒超声在诊断主动脉窦瘤破裂中的应用

目的:探讨经胸及经食道彩色多普勒超声心动图在诊断主动脉窦瘤破裂的应用价值。方法:用彩色多普勒超声心动图检查有级以上心脏杂音,诊断为主动脉窦瘤破裂合并室间隔缺损(VSD)及主动脉瓣关闭不全的患者,共16例。结果:16例主动脉窦瘤破裂患者中,右冠窦瘤破裂13例,无冠窦瘤破裂3例,合并室间隔缺损9例,合并主动脉瓣脱垂3例,合并肺动脉瓣狭窄及动脉导管未闭各1例。结论:彩色多普勒超声心动图可以直观地显示主动脉窦瘤的有无及其破裂口的大小和数目,还可以对主动脉窦瘤破裂合并心脏畸形的类型作出诊断,很有意义。     

Initial clinical experience of real-time three-dimensional echocardiography in neonates with isolated congenital ductus arteriosus aneurysm

BACKGROUND: Congenital ductus arteriosus aneurysm (DAA) was considered rare but potentially fatal abnormality, often followed by surgical intervention after careful evaluation. This prospective study used real-time three-dimensional echocardiography (RT3DE) to assist in evaluation of neonatal DAA. METHODS: A total of 1390 full-term neonates were enrolled in this study between 2002 and 2003. They received two-dimensional echocardiographic (2DE) screening and periodic follow-up. RT3DE was performed selectively for newborns with DAA. RESULTS: DAA were detected in 116 (8.34%) newborns using 2DE. Maximum diameter of the DAAs ranged from 6.8 to 14.0 mm (8.2+/-1.1 mm). None of the cases were symptomatic or had complications related to DAA. There were no significant differences in sex and gestational age between the newborns with and without DAA. Neonates with DAA had a higher birth body weight and a higher incidence of large-for-gestational-age (P<0.05). RT3DE provided instant, consistent and reliable 3D images of DAA and its related structures and allowed for more rapid examination times and reduction of baby wait times. CONCLUSIONS: Congenital DAA is as common as has been previously reported. RT3DE is useful in assisting evaluation of DAA. Preferred images of DAA were typically visualized in the high parasternal short-axis view before the third day of life. Routine use of RT3DE is suggested to enhance assessment of neonates with DAA detected by 2DE.     

17例主动脉夹层动脉瘤临床病理分析

对１７例主动脉夹层动脉瘤的临床病理进行了分析。其中１１例合并高血压（高血压组）；６例合并马凡氏综合征（马凡氏组）。前者年龄较大，病起常有剧烈的心前区疼痛，夹层多属Ｉ或ＩＩＩ型，常合并严重的动脉粥样硬化。内破口常在升主动脉或胸主动脉；患者常因夹层破裂引起出血性休克而死亡。后者年龄较轻，病程时间较长，均有升主动脉夹层动脉瘤形成，破口常与夹层腔融合。主动脉无或仅有轻度动脉粥样硬化，病变常累及主动脉窦和主动脉瓣，患者常因顽固性心力衰竭而死亡。对主动脉夹层动脉瘤的形成和主要病理改变，结合文献进行了分析、讨论。     

Approach to the dilated aortic root

PURPOSE OF REVIEW: The dilated aortic root is often completely asymptomatic and found incidentally on routine imaging studies such as chest radiograph, echocardiography, chest computed tomography, or magnetic resonance imaging. The dilated aortic root may be associated with underlying aortic valve abnormalities as seen with bicuspid aortic valve. It may also lead to the awareness of important underlying connective tissue disorders like the Marfan syndrome. It is imperative that the dilated aortic root be observed carefully over time with serial imaging studies and that timely resection of the aneurysm be carried out before catastrophic complications such as aortic dissection, aortic rupture, or congestive heart failure from aortic insufficiency occur. RECENT FINDINGS: In recent years, the advent of molecular genetics has heightened awareness of familial aortic disease such as the Marfan syndrome, bicuspid aortic valve disease, and hereditary aortic aneurysm and dissection. In addition to hypertension and inflammatory aortic disease, these hereditary aortopathies are important to consider in the evaluation of patients with a dilated aorta and have implications for screening of the relatives of the patient with aortic aneurysm. SUMMARY: Because there is often uncertainty regarding the dilated aortic root, this review will summarize the approach to diagnosis, evaluation, and management of aortic root aneurysms. Clinical features, diagnostic approaches, screening of relatives, and long term follow-up will be highlighted.     

Clinical usefulness of transesophageal Doppler echocardiography

The usefulness of transesophageal Doppler echocardiography (TEE) was assessed in patients with various cardiovascular diseases including 15 patients with dissecting aortic aneurysm (DAA), two with thoracic aneurysm, 16 with ischemic heart disease and 14 with acquired valvular diseases. In dissecting aortic aneurysms, TEE provided clear images of the intimal flaps even in the aortic arch and descending aorta in which clear images could not be obtained by conventional external Doppler echocardiography. The entry site was detected in 11 of the 15 (73%) cases using TEE, but in only three of the 15 cases using conventional Doppler technique. In two cases of true aortic aneurysms, TEE provided clear images of the aneurysm in the descending thoracic aorta, which was discriminated precisely from DAA. In valvular disease, all four valves (aortic, pulmonary and atrio-ventricular valves) were easily observed without disturbance by any other tissues using a transesophageal approach. In addition, valve aneurysms in the posterior mitral leaflets were detected using TEE in two cases. In two cases of mitral stenosis, a thrombus was observed in the left atrial appendage. These findings were confirmed during surgery, but could not be obtained by the conventional external studies. In 16 cases, TEE was performed during aorto-coronary bypass surgery under general anesthesia. In two of these cases, left ventricular assist devices were applied after surgery. In these cases, where conventional Doppler echocardiography was not applicable, cardiac function could be monitored by TEE. Thus, TEE is useful not only in evaluating morphological function in the cardiovascular system but also in monitoring cardiac hemodynamics during and after heart surgery.     

Obstetric complications in Marfan syndrome

OBJECTIVE: To investigate the obstetric maternal and neonatal outcome of pregnancy in women with Marfan syndrome. METHODS: Retrospective observational multi-center study based on congenital heart disease registry. RESULTS: Sixty-three of the 122 enrolled women with Marfan syndrome had been pregnant 142 times (including 111 pregnancies>20 weeks, 28 (20%) miscarriages and 3 elective abortions). In 40% of all completed pregnancies an obstetric and/ or neonatal complication occurred. The most important complications were an increased percentage of premature deliveries (n=17, 15%) mainly due to preterm premature rupture of membranes and cervical incompetence and a markedly increased combined fetal and neonatal mortality of 7.1%. An obstetric and/or neonatal complication occurred in a similar proportion of pregnancies in women with a diagnosis of Marfan syndrome before pregnancy versus women with a diagnosis of Marfan syndrome after pregnancy (39% vs. 41%, p=0.85, respectively). CONCLUSION: In addition to cardiovascular complications, pregnancy in women with Marfan syndrome is associated with a high rate of premature deliveries, preterm premature rupture of membranes and increased mortality in the offspring.     

Phenotypic alteration of vascular smooth muscle cells precedes elastolysis in a mouse model of Marfan syndrome

Marfan syndrome is associated with early death due to aortic aneurysm. The condition is caused by mutations in the gene (FBN1) encoding fibrillin-1, a major constituent of extracellular microfibrils. Prior observations suggested that a deficiency of microfibrils causes failure of elastic fiber assembly during late fetal development. Mice homozygous for a targeted hypomorphic allele (mgR) of Fbn1 revealed a predictable sequence of abnormalities in the vessel wall including elastic fiber calcification, excessive deposition of matrix elements, elastolysis, and intimal hyperplasia. Here we describe previously unrecognized concordant findings in elastic vessels from patients with Marfan syndrome. Furthermore, ultrastructural analysis of mgR mice revealed cellular events that initiate destructive changes. The first detectable abnormality was an unusually smooth surface of elastic laminae, manifesting the loss of cell attachments that are normally mediated by fibrillin-1. Adjacent cells adopted alteration in their expression profile accompanied by morphological changes but retained expression of vascular smooth muscle cell markers. The abnormal synthetic repertoire of these morphologically abnormal smooth muscle cells in early vascular lesions included elastin, among other matrix elements, and matrix metalloproteinase 9, a known mediator of elastolysis. Ultimately, cell processes associated with zones of elastic fiber thinning and fragmentation. These data suggest that the loss of cell attachments signals a nonproductive program to synthesize and remodel an elastic matrix. This refined understanding of the pathogenesis of vascular disease in Marfan syndrome will facilitate the development of therapeutic strategies.     

Azygous vein aneurysm: CT scan follow-up

We present a case of azygous vein aneurysm diagnosed by dynamic contrast-enhanced CT scan. The patient refused surgical resection, although it was offered as one of the treatment options due to theoretical risks of rupture and pulmonary embolism. The patient has been managed conservatively for 4 years with no untoward outcomes. The aneurysm showed no change in size or thrombus formation. The patient remained asymptomatic. Our case showed that conservative management may be a reasonable approach for asymptomatic azygous vein aneurysm with no thrombus formation.     

Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome

Marfan syndrome is a disorder of the connective tissue that is inherited in an autosomal-dominant fashion and that is caused by mutations in the gene coding for fibrillin-1, FBN1. Although complications of the syndrome may involve the eye, the lung and the skeleton, the high mortality of untreated cases results almost exclusively from cardiovascular complications, including aortic dissection, rupture and mitral valve regurgitation. The multiorgan involvement of many of these syndromes requires multidisciplinary expert centers that can increase the average life expectancy of affected patients from only 32 years to over 60 years. The present article both reviews classical standards of managing cardiovascular manifestations and highlights the surgical approach for aortic and mitral valve surgery in Marfan patients.     

Caring for a Marfan patient with cardiovascular complications.

The Marfan syndrome is a heritable disorder of connective tissue associated with characteristic abnormalities of the skeletal, ocular and cardiovascular systems. Common cardiovascular manifestations of this syndrome are mitral valve prolapse with mitral regurgitation and dilatation of the ascending aorta resulting in aortic insufficiency, dissection, aneurysm and/or rupture. Although the prognosis for a patient with the Marfan syndrome is significantly more favorable than it was ten years ago, the cardiovascular complications continue to greatly reduce life expectancy. This article presents an overview of the Marfan syndrome including: history and epidemiology, clinical manifestations, diagnostic criteria, surgical intervention and follow-up. A case study is outlined which focuses on priority nursing diagnoses and a plan of care.     

Silent patent ductus arteriosus aneurysm

Ductus arteriosus aneurysm, a rare and potentially fatal condition, has been reported as a complication after surgical ductus arteriosus closure. Its spontaneous appearance as a septic complication, which was common in the presurgical and preantibiotic era, has been rarely reported in the contemporary literature. Persistence of silent ductus arteriosus in healthy children and adults is a frequent condition that currently has an increasing diagnostic possibility due to the availability of more accurate investigative methods, especially echocardiography. We report the case of a 1-year-old child, in whom no previous heart disease was known, who developed a giant aneurysm of the ductus arteriosus during a staphylococcal infection. This complication appeared after craniotomy for emptying an accidental subdural hematoma. This report associates the persistence of ductus arteriosus with a complication considered rare, which has a rapidly fatal evolution.     

Thrombosed aneurysm of the left sinus of Valsalva

The authors present the case of a 57-year-old asymptomatic woman, in whom a large left coronary sinus of Valsalva aneurysm was incidentally diagnosed on a routine echocardiogram in 1998. The case was initially presented to cardiac surgery consultants, and since there were no signs of rupture and the patient was asymptomatic, it was decided to keep her in close clinical and echocardiographic follow-up. Eight years later, and with no clinical manifestation in the meantime, a transesophageal echocardiogram (TEE) showed that the aneurysm was filled with swirling spontaneous echo contrast ("smoke") overlying a thrombus, which was not detected by transthoracic echocardiography. The patient then underwent surgical treatment with aortic root and aortic valve replacement and coronary reimplantation, with an excellent result. Although the need for early surgical intervention in patients with ruptured sinus of Valsalva aneurysms is well established, the optimal management of an asymptomatic, unruptured aneurysm is less clear, due to the absence of a precise natural history. The follow-up of our patient clearly demonstrated that it is mandatory to assess unruptured sinus of Valsalva aneurysms by TEE, particularly to exclude thrombotic complications. Such complications are one of the possible paths of the natural history of unruptured sinus of Valsalva aneurysms, and support the indication for early surgical treatment to avoid future complications.     

Morphologic and angiographic features of coronary plaque rupture detected by intravascular ultrasound

OBJECTIVES: This study was designed to report the clinical and angiographic correlates of plaque rupture detected by intravascular ultrasound (IVUS). BACKGROUND: Acute coronary syndromes result from spontaneous plaque rupture and thrombosis. METHODS: We report 300 plaque ruptures in 257 arteries in 254 patients. Plaque ruptures were detected during pre-intervention IVUS. Standard clinical, angiographic, and IVUS parameters were collected and/or measured. One lesion per patient was analyzed. RESULTS: Multiple ruptures were observed in 39 of 254 patients (15%), 36 in the same artery. Plaque rupture occurred not only in patients with unstable angina (46%) or myocardial infarction (MI, 33%), but also stable angina (11%) or no symptoms (11%). The tear in the fibrous cap could be identified in 157 of 254 patients; 63% occurred at the shoulder of the plaque and 37% in the center of the plaque. Thrombi were more common in patients with unstable angina or MI (p = 0.02) and in multiple ruptures (p = 0.04). The plaque rupture site contained the minimum lumen area (MLA) site in only 28% of patients; rupture sites had larger arterial and lumen areas and more positive remodeling than MLA sites. Intravascular ultrasound plaque rupture strongly correlated with complex angiographic lesion morphology: ulceration in 81%, intimal flap in 40%, thrombus in 7%, and aneurysm in 7%. CONCLUSIONS: Plaque ruptures occur with varying clinical presentations, strongly correlate with angiographic complex lesion morphology, may be multiple, and usually do not cause lumen compromise.     

Evolution of ventricular outpouching through the fetal and postnatal periods: Unabating dilemma of serial observation or surgical correction

Ventricular outpouching is a rare finding in prenatal sonography and the main differential diagnoses are diverticulum, aneurysm, and pseudoaneurysm in addition to congenital cysts and clefts. The various modes of fetal presentation of congenital ventricular outpouching include an abnormal four-chamber view on fetal two-dimensional echocardiogram, fetal arrhythmia, fetal hydrops, and pericardial effusion. Left ventricular aneurysm (LVA)/nonapical diverticula are usually isolated defects. Apical diverticula are always associated with midline thoracoabdominal defects (epigastric pulsating diverticulum or large omphalocele) and other structural malformations of the heart. Most patients with LVA/congenital ventricular diverticulum remain clinically asymptomatic but they can potentially give rise to complications such as ventricular tachyarrhythmias, systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation. The treatment of asymptomatic LVA and isolated congenital ventricular diverticulum is still undefined. In this review, our aim is to outline a systematic approach to a fetus detected with ventricular outpouching. Starting with prevalence and its types, issues in fetal management, natural course and evolution postbirth, and finally the perpetual dilemma of serial observation or surgical correction is discussed.     

Plaque rupture with severe pre-existing stenosis precipitating coronary thrombosis. Characteristics of coronary atherosclerotic plaques underlying fatal occlusive thrombi.

Ruptured atheromatous plaques were identified by step-sectioning technique as responsible for 40 of 51 recent coronary artery thrombi and 63 larger intimal haemorrhages. The degree of pre-existing luminal narrowing at the site of rupture was decisive for whether plaque rupture caused occlusive thrombosis or just intimal haemorrhage. If the pre-existing stenosis was greater than 90% (histologically determined) then plaque rupture nearly always caused occlusive thrombosis. Clearly indicating the primary role of plaque rupture in thrombus formation were the frequent finding of plaque fragments deeply buried in the centre of the thrombus and the nature of the thrombus at the site of rupture where it consisted predominantly of platelets. Thus, a severe chronic stenosis seems to be a prerequisite for occlusive thrombus formation, but the thrombotic process itself is triggered by an acute intimal lesion.