Pulmonary and aortic valve endocarditis in an adult patient with silent patent ductus arteriosus

Pulmonary and aortic valve endocarditis are uncommon especially in an adult patient with patent ductus arteriosus. A 27-year-old woman diagnosed with pulmonary and aortic valve endocarditis underwent surgical treatment. Here, we report our clinical and surgical experience in treating a case of double valve endocarditis with clinically silent patent ductus arteriosus.     

Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.     

Systemic and pulmonary embolization in a patient with patent ductus arteriosus.

We report on a patient with unrecognized patent ductus arteriosus and infective endocarditis of the aortic valve with septic embolization to the right pulmonary artery and the coronary tree.     

Patent ductus arteriosus in adult patients with aortic valvular disease: The importance of routine screening for a left-to-right shunt at cardiac catheterization

Two patients, catheterized primarily for the preoperative assessment of clinically apparent aortic stenosis and insufficiency, were found to have a patent ductus arteriosus as well at cardiac catheterization. The association of aortic valve disease and patent ductus arteriosus is reviewed, and the importance of making the diagnosis prior to aortic valve replacement is stressed, emphasizing the routine use of the hydrogen-platinum electrode system.     

Patent ductus arteriosus in adult patients with aortic valvular disease: the importance of routine screening for a left-to-right shunt at cardiac catheterization.

Two patients, catheterized primarily for the preoperative assessment of clinically apparent aortic stenosis and insufficiency, were found to have a patent ductus arteriosus as well at cardiac catheterization. The association of aortic valve disease and patent ductus arteriosus is reviewed, and the importance of making the diagnosis prior to aortic valve replacement is stressed, emphasizing the routine use of the hydrogenplatinum electrode system.     

Functional Critical Aortic Stenosis with Transient Retrograde Flow in a Neonate with Left Diaphragmatic Hernia

We report a neonate with left congenital diaphragmatic hernia and severe left ventricular dysfunction, in whom the blood flow in the transverse arch and its branches was supported in a retrograde fashion by patent ductus arteriosus. There was only minimal antegrade flow across the aortic valve and hemodynamic physiology resembled critical aortic stenosis, necessitating the immediate use of prostaglandin E1 infusion to maintain the patent ductus arteriosus.     

Long-term survival in preductal atresia of the aortic arch

Atresia of the aortic arch is distinguished from complete interruption of the aorta by the presence of a fibrotic strand bridging the 2 discontinuous segments of aorta. Most patients with aortic arch atresia have associated intracardiac defects. There is a high neonatal mortality in this condition, with 95% of the patients dying in the first year of life. The case described herein is the first long-term survivor with atresia of the aortic arch, a patent ductus arteriosus, and an atrial septal defect to reach adulthood. In this patient an extensive collateral network existed in spite of the presence of a patent ductus arteriosus.     

Two-dimensional echocardiographic features of interruption of the aortic arch

The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized from an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.     

Transesophageal echocardiographic monitoring of interventional cardiac catheterization in children

Transesophageal echocardiography was used prospectively in 22 children scheduled for interventional cardiac catheterization (9 with pulmonary valvuloplasty, 5 with aortic valvuloplasty, 1 with pulmonary angioplasty, 2 with aortic angioplasty, 2 with patent ductus arteriosus occlusion and 3 with Mustard baffle dilation) to determine its potential value as a monitoring technique. The patients ranged in age from 0.9 to 14.6 years (mean 5.4) and in weight from 9.5 to 49.2 kg (mean 21.1). Studies were completed in all patients without complications. Preintervention studies provided important new information in two patients, leading to cancellation of the planned procedure. Major contributions of transesophageal monitoring included 1) a real time assessment of catheter placement across either atrioventricular valve and the aortic valve during balloon valvuloplasty; 2) immediate assessment of aortic valve and aortic wall morphology during balloon dilation; and 3) detailed morphologic and hemodynamic information together with enhanced catheter guidance during Mustard baffle dilation. After pulmonary valvuloplasty, partial chordal rupture of the tricuspid valve was documented in one patient. In two patients, balloon catheter position was modified according to the transesophageal findings. The assessment of changes in pulmonary valve morphology and transcatheter occlusion of a patent ductus arteriosus was not enhanced by single-plane transesophageal monitoring. Pulsed wave Doppler studies contributed additional information in the assessment of immediate hemodynamic changes after interventional procedures. Transesophageal echocardiography is a new important guiding and monitoring technique during interventional cardiac catheterization procedures in children. It can provide additional real time imaging information, immediate identification of complications and assessment of hemodynamic changes.     

Transcatheter coil embolization for patent ductus arteriosus in the elderly: Report of a case and review of the published work

Patent ductus arteriosus is the third most common congenital cardiovascular anomaly, however, it is rarely found in the elderly. We describe a case of patent ductus arteriosus in a 72-year-old woman in whom patent ductus arteriosus was successfully managed by transcatheter coil embolization. The patient had been diagnosed with a heart murmur for the first time 1 year earlier at the age of 71. She was asymptomatic but a continuous murmur was heard. Cardiac catheterization revealed migration of a catheter from the main pulmonary artery into the descending aorta through a patent ductus arteriosus and a significant step-up of oxygen saturation in the main pulmonary artery with a pulmonary-to-systemic flow ratio of 1.68. Aortograms demonstrated a communication between the aorta and the pulmonary artery through a patent ductus arteriosus with a minimal diameter of 3.7 mm. Transcatheter coil embolization of the patent ductus arteriosus was successfully carried out with two 0.052-inch-diameter Gianturco coils. Doppler echocardiographic study confirmed no residual shunt in the main pulmonary artery after the procedure. Non-surgical transcatheter occlusion using coil embolization appears to be an effective and minimally invasive technique for treatment of patent ductus arteriosus in the elderly.     

False aneurysm complicating ligation of patent ductus arteriosus operated on successfully

Postoperative aneurysm of ductus arteriosus is a rare complication but may be lethal without treatment. It is less frequent than spontaneous aneurysm of ductus arteriosus. We report the case of 5 years-old girl who underwent a ligation of patent ductus arteriosus complicated, 6 months later, with a false aneurysm of ductus arteriosus and endocarditis of the ductus and the aortic valve. The diagnosis of the aneurysm was suspected on the anteroposterior chest X-ray which showed a left superior mediastinal opacity and confirmed by echocardiography. Through bilateral thoracotomy, an anevrismorraphy and aortic repair was carried out without problem. The postoperative course was unremarkable.     

Transcatheter treatment of "pulmonary artery hypertension" due to patent ductus arteriosus and pulmonary artery stenosis

The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was found to complicate the hemodynamic picture of a moderate-to-large patent ductus arteriosus (QP/QS, 1.7:1), by causing pulmonary hypertension (mean pressure, 65 mmHg) and left-to-right pulmonary flow imbalance. Both lesions were treated in a single procedure of right pulmonary artery stenting and patent ductus arteriosus closure, after which the pulmonary artery pressure significantly decreased (mean, 35 mmHg). In our opinion, a thorough hemodynamic evaluation followed by pulmonary angiography should be mandatory before proceeding to patent ductus arteriosus closure in the adult patient who has "hypertensive" ductus, in whom possible associated malformations can be missed due to a poor echocardiographic window.     

54例主动脉缩窄的外科治疗

我院 1984年 6月至 1998年 6月 ,共收治 54例主动脉缩窄病人。男性 2 7例 ,女性 2 7例 ,平均年龄 15.8岁 ( 5个月～ 39岁 )。其中婴儿型 18例 ,成人型 36例 ,合并动脉导管未闭 33例 ,室间隔缺损 2 0例 ,房间隔缺损 4例 ,主动脉瓣二瓣化畸形 4例 ,主动脉瓣下狭窄 3例 ,二尖瓣狭窄 2例 ,二尖瓣脱垂 1例。其中 39例进行狭窄处补片扩大成形术 ,5例进行狭窄处切除主动脉端端吻合术 ,3例进行了狭窄处切除人工血管移植术 ,4例进行了人工血管转流手术 ,3例用主肺动脉内隧道的方法 ,实现了升主动脉 降主动脉的连接 ,同时行室缺修补。手术死亡 1例 ( 1.85% )。远期随访死亡 3例 ( 5.6% ) ,其余效果良好     

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion.We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.     

Clinical and echocardiographic characteristics of scimitar syndrome

Seven patients with scimitar syndrome underwent retrospective clinical and echocardiographic examination. The findings were: respiratory infection with dyspnea on moderate exercise in 90%, scimitar sign in four (57%), dextrocardia in five (71%), and interatrial septal defect in five (71%), one of whom had patent ductus arteriosus. Overall, two patients had patent ductus arteriosus: one also had aortic coarctation and the other, a bicuspid aortic valve. Dilation of the right cavities was found in five (71%) and blunt edge in five (71%). In two patients, anomalous drainage was into the right atrium; in another two, into the inferior vena cava; and in three, towards the junction of the right atrium and the inferior vena cava. In three patients, drainage was obstructed. Six patients with cardiac abnormalities proceeded to surgery. Scimitar syndrome is a rare entity. In the large majority of patients (86%), diagnosis and follow-up can be performed echocardiographically.     

Aortic thrombus after coil occlusion of a type E patent ductus arteriosus

A 5-year-old girl was referred to our institution for closure of a silent patent ductus arteriosus. Cardiac catheterization revealed a tiny-to-small patent ductus arteriosus of the elongated, conical type (Type E). Coil occlusion was performed with a Gianturco coil, 0.035-4 cm(-3) mm. A follow-up echocardiogram showed a very small residual ductal shunt and a moderate-sized thrombus at the aortic end of the ductus arteriosus. The patient remained asymptomatic.     

Primary bronchomalacia and patent ductus arteriosus: simultaneous surgical correction in an infant.

We report the clinical course of a 6-month-old girl with recurrent infection of the left lung, persistent wheezing, and a suspected congenital heart anomaly (patent ductus arteriosus. Chest radiography revealed hyperinflation and slight inflammation of the left lung. Tracheobronchoscopy and left-sided bronchography showed a collapsed segment of the left main bronchus, 3 cm long. Computed tomography confirmed hyperinflation of the left lung and atelectasis of the superior lobe. There were no signs of extramural compression. Color-flow Doppler echocardiography confirmed the suspicion of patent ductus arteriosus. To the best of our knowledge, there is no other report in the literature of a patient with this combination of anomalies. After receiving 2 weeks of antibiotic treatment, the patient underwent surgical repair The patent ductus arteriosus was closed by means of a triple-ligature procedure, and during the same operation a bronchopexy was performed, securing the left main bronchus to the closed ductus tissue by means of sutures. There have been no complications in the postoperative period. Clinical follow-up, as well as echocardiography and bronchoscopy, have yielded normal results 14 months after surgery.     

A neonate with mitral stenosis due to accessory mitral valve,ventricular septal defect,and patent ductus arteriosus: changes in echocardiographical findings during the neonatal period

 A female neonate with mitral stenosis due to accessory mitral valve with ventricular septal defect and patent ductus arteriosus is described. She was referred to our hospital because of neonatal asphyxia. Asphyxia was improved by ventilator support, but rapid deterioration of respiration with pulmonary congestion and hemorrhage appeared 8 days after birth. Echocardiography revealed an accessory mitral valve attached to the anterior mitral leaflet with a perimembranous ventricular septal defect and patent ductus arteriosus. Although there were no echocardiographical findings indicating mitral stenosis on admission, the mitral stenosis blood flow patterns were detected by color and pulsed Doppler examination performed on the eighth day after admission. Transaortic resection of accessory mitral valve tissue was performed with patch closure of the ventricular septal defect and ligation of the ductus arteriosus 35 days after birth. After operation, pulmonary congestion and hemorrhage were improved. Postoperative echocardiography showed complete resection of the accessory mitral valve and no mitral insufficiency. We concluded that the combination of the accessory mitral valve and left-to-right shunt due to ventricular septal defect or patent ducturs arteriosus might have led to a critical hemodynamic condition due to relative mitral stenosis in the neonatal period with the decrease in pulmonary vascular resistance. Received: May 30, 2002 / Accepted: September 6, 2002 Correspondence to T. Ito     

Truncus arteriosus malformation: a spectrum including fourth and sixth aortic arch interruptions

Previous explanations of the pathogenesis of truncus arteriosus malformation have emphasized absence of conotruncal ridges, absence of pulmonary conus, or fusion of ventricular outflow streams. These concepts explain the persistence of the single semilunar valve and outflow vessel, but have not elucidated the significance of many associated anatomic lesions. We studied the 19 patients with truncus arteriosus malformation listed in the autopsy files of The Johns Hopkins Hospital whose hearts were available for review. The patients ranged in age from one day to 30 years, and included 12 males. All hearts showed a single semilunar valve with three or four cusps, and a high ventricular septal defect. In 13 patients, the aorta was larger in diameter than the pulmonary artery, and no remnant of the ductus arteriosus was present (interruption of the embryonic sixth arch). In one patient, the aorta was larger than the pulmonary artery, and a small, patent ductus arteriosus was present. In two patients, the pulmonary artery was larger than the aorta, and the aortic arch was interrupted. The remaining three cases could not be fully evaluated for the status of the ductus arteriosus and size of the great arteries. We tabulated the number of associated anatomic lesions which were unexplained by each of the three pathogenetic hypotheses. The fused outflow stream hypothesis, which explains truncus arteriosus malformation as the result of a maldirection of ventricular outflow streams so that the separation of two semilunar valves is prevented, was superior at the 0.05 level of significance. This concept explains the spectrum of great vessel patterns in which flow is dominant into either the sixth or fourth embryonic aortic arch, permitting early disappearance of unperfused segments of the aortic arches, and is commonly seen at autopsy as either an interrupted aorta or an absent ductus arteriosus.     

New serial contrast technique for assessment of left to right shunting patent ductus arteriosus in the neonate

A new serial echocardiographic contrast technique for detection of patent ductus arteriosus has been developed and validated by clinical course (33 infants), surgical observations (13 infants) and autopsy observations (4 infants). A left to right shunting patent ductus arteriosus was demonstrated in 30 of 33 prospectively studied premature infants (mean weight 1,371 g) using this new contrast method. The demonstration of left to right ductal shunting was accomplished by hand injection of a nonviscous material (saline solution, 5 percent dextrose in water or the patient's own blood) through an umbilical arterial catheter placed with its tip located above the diaphragm during recording of a suprasternal notch echocardiogram. In positive studies, both the transverse aortic arch and right pulmonary artery were opacified. If no left to right shunt existed, only the transverse aortic arch was opacified.This serial study helped elucidate the natural history of patent ductus arteriosus. Twenty-four of the 30 patients with a positive study initially had a left to right shunting patent ductus arteriosus without an audible murmur. Three of these patients with silent patent ductus arteriosus later had congestive heart failure and two required operative ligation of the patent ductus. The high rate of detection of silent patent ductus arteriosus and its bedside confirmation when suspected are important in the serial management of critically ill newborns with this condition. The test is safe and sensitive and it is useful for early demonstration of silent patent ductus arteriosus, for clarifying the origin of murmurs and for confirming spontaneous, operative or pharmacologie closure of a patent ductus arteriosus.