Aortopulmonary Artery Fistula: Ruptured Aneurysm of the Distal Aortic Arch into the Pulmonary Artery

Aortopulmonary artery fistula is uncommon, but the clinical outcome is often lethal. A 76‐year‐old man with a history of acute thoracic aortic dissection 6 years previously was admitted with dyspnea. A chest x‐ray showed pleural effusion and pulmonary congestion. Transthoracic echocardiography revealed preserved systolic function, but continuous and abnormal flow from the distal aortic arch into the pulmonary artery (PA). Transesophageal echocardiography (TEE) in the Doppler color‐flow mode demonstrated a left‐to‐right shunt between a large distal aortic arch aneurysm and the left PA via an aortopulmonary fistula and a pressure gradient across the shunt of 56 mmHg. Contrast‐enhanced computed tomography showed that the aneurysm compressed the PA. Aortography also revealed a large distal aortic arch aneurysm and almost simultaneous contrast enhancement of the aorta and the PA. Right‐heart catheterization showed a significant increase in oxygen saturation between the right ventricle and the PA. A left‐to‐right shunt due to a distal aortic arch aneurysm rupturing into the left PA was diagnosed based on these findings. TEE was very helpful in confirming the presence and precise location of the fistula.     

Ductus-associated proximal pulmonary artery stenosis in patients with right heart obstruction

Proximal pulmonary artery stenosis is a common acquired lesion in infants treated for congenital heart disease. We hypothesized that a large number of stenoses develop at the site of ductal insertion in patients with right ventricular outflow tract obstruction (RVOTO) and that these patients are at risk for developing hypoplasia of the ipsilateral pulmonary artery. The surgical and cardiac catheterization databases at our institution during the years 1988-2000 were searched for all patients under 1 year of age carrying a diagnosis of pulmonary atresia with intact ventricular septum (PA), tetralogy of Fallot (TOF) or pulmonary stenosis (PS), yielding 700 patients (62 PA, 373 TOF, 265 PS). The cardiac catheterization database was also searched for all patients with any diagnosis under 1 year of age found at catheterization to have proximal pulmonary artery stenosis. Proximal pulmonary artery stenosis associated with the ductal insertion site was diagnosed at catheterization in 33 infants (18 with PA, 5 with TOF, 6 with PS, 4 other diagnoses). This represents 29% of patients with PA, 1% with TOF and 2% with PS. Among patients with RVOTO and ductal insertion site-associated stenosis, there was a high prevalence (59%) of associated distal pulmonary arterial hypoplasia, defined as diameter of the stenosed vessel at first distal branch < or = 80% the diameter of the contralateral vessel. Symptomatology failed to identify this lesion; therefore, a high index of suspicion is necessary if proximal pulmonary artery stenosis is to be detected early in these patients.     

Dynamic Pulmonary Artery Obstruction Causing Right Ventricular Failure after Cardiac Transplantation in a Patient with Mustard–Senning Corrected d‐Transposition of the Great Arteries

Patients with congenital d‐transposition of the great arteries (d‐TGA) undergoing palliative atrial baffle surgery in infancy often develop systemic ventricular failure in adulthood. If they undergo cardiac transplantation, they are prone to morphologic right ventricular (RV) failure secondary to severe pulmonary hypertension as a result of systemic ventricular failure. We report a case of a patient with d‐TGA and biventricular ventricular failure requiring heart transplantation (HT) that developed RV failure postoperatively because of dynamic pulmonary artery (PA) obstruction at the anastomotic site of PA. Obstruction at the site of PA anastomosis due to torsion or redundancy of the donor or recipient PA is a rare but treatable cause of postoperative RV failure. In this case, rapid identification of the etiology of RV failure and implementation of corrective therapies before the development of end‐organ dysfunction, resulted in complete RV recovery and normal allograft function. This case represents the first known report of dynamic PA anastomoticobstruction resulting in RV failure after HT that was corrected with pulmonary arterioplasty, and RV assist device resulting in complete recovery.     

Aortic Tumor or Mobile Thrombus?

Isolated large mobile mass in the thoracic aorta can be due to thrombus or, rarely, aortic tumor. We report the case of a 61‐year‐old man with no history of medical problems presenting with neurologic deficits and in whom a large mobile echogenic mass in the distal aortic arch was found with transesophageal echocardiography. Given his few cardiovascular risk factors and absence of other systemic symptoms, he received anticoagulant therapy. Subsequent resolution of the aortic mass suggested a diagnosis of thrombus. This case illustrates an unusual manifestation of aortic arch atherosclerosis and underscores the utility of transesophogeal echocardiography for patients with ischemic stroke. (Echocardiography 2010;27:E21‐E22)     

Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

In cases of pulmonary atresia with ventricular septal defect (PA‐VSD), coronary‐pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA‐VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.     

Right Aortic Coarctation and Ventricular Septal Defect: An Unusual Cause of Tracheal Compression in Infancy

Objective. Acyanotic congenital heart diseases may occasionally present with tracheobronchial obstruction. Increased pulmonary blood flow against a high‐resistance pulmonary bed may create significant pulmonary artery dilation. Methods. We report an unusual case of ventricular septal defect and right aortic arch coarctation, complicated with distal tracheal compression secondary to a pincer effect created by a right aortic arch and a massively dilated pulmonary artery. Results. High index of suspicion is required to anticipate tracheobronchial compression in acyanotic congenital heart diseases. Conclusion. Fiberoptic bronchoscopy is an invaluable tool to assess for tracheal compression relief following surgical repair and to identify tracheomalacia prior to extubation.     

Aortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course

PHACE is a rare congenital neurocutaneous syndrome where posterior fossa malformations, hemangiomas, cerebrovascular anomalies, aortic arch anomalies, cardiac defects, and eye abnormalities are variably associated. We describe the prenatal detection and the postnatal course of a child with PHACE syndrome with a unique type of aortic arch anomaly consisting of proximal interruption of the aortic arch and persistence of the fifth aortic arch. The fifth aortic arch represented in this case a vital systemic‐to‐systemic connection between the ascending aorta and the transverse portion of the aortic arch allowing adequate forward flow through the aortic arch without surgical treatment.     

Stent Implantation of the Arterial Duct in Newborns with a Truly Duct‐Dependent Pulmonary Circulation: A Single‐Center Experience with Emphasis on Aspects of the Interventional Technique

Introduction: Ductal stenting for pulmonary blood supply in newborns with cyanotic congenital heart disease (CHD) might be a low risk and safe alternative to the surgical aorto‐to‐pulmonary artery (AP) shunt in dual‐source lung perfusion. Ductal stenting in truly duct‐dependent pulmonary circulation has not been evaluated. Methods: Prospective interventional and clinical follow‐up trial. Ductal stenting based on variable access sites, a 2‐wire technique when crossing a tortuous ductus, and use of premounted coronary stents. Primary outcome measures were procedural success and complication rates presented as early and mid‐term results. Results: From 2003–2009, 58 duct‐dependent newborns underwent ductal stenting; 27 of them were truly duct dependent, 20 had pulmonary atresia (PA)/ventricular septum defect or complex CHD, 4 had PA/intact ventricular septum, 2 had PA with Ebstein anomaly, and 1 had PA with tricuspid atresia. Ductal stenting was performed without procedure‐related mortality; 3 of 27 required an acute surgical AP‐shunt (stent migration in 1, acute duct obstruction in 2). During mid‐term follow‐up, 4 of 24 needed an AP‐shunt and two others stent redilation. Three patients died prior to follow‐up surgery (1 unexpectedly at home and 2 due to syndromatic disease). Fifteen patients received staged univentricular palliation, 8 had a biventricular repair, and 1 is awaiting follow‐up operation. Conclusion: Ductal stenting is a feasible, safe, and effective palliation in newborns with truly duct‐dependent pulmonary circulation irrespective of duct morphology. Vasucular access from various locations is important for technical success rate. Ductal stenting is a minimally invasive procedure to achieve adequate pulmonary artery growth for subsequent palliative or corrective surgery. (J Interven Cardiol 2010;23:581–588)     

High Long‐term Morbidity in Repaired Aortic Coarctation

Objective. The objective of this study was to assess late morbidity after repair of aortic coarctation and its association with residual aortic arch obstruction. Design and Setting. This is an observational cohort study of 133 patients who underwent surgical repair during 1965–1985. Echocardiography, bicycle exercise testing, 24‐hour ambulatory blood pressure monitoring, and magnetic resonance imaging/computerized tomography scan of the thoracic aorta were performed. The setting of this study was a tertiary referral center. Patients. Among 156 survivors, 133 (84 men) accepted study participation. Median age (range) was 10 (0.1–40) years at repair and 44 (26–74) years at follow‐up. Outcome Measures. Outcome measures used are prevalence of previous cardiovascular reinterventions, current cardiac and valvular function, exercise capacity, blood pressure levels at rest and during exercise, and presence of recurrent or residual aortic arch obstruction and/or aortic aneurysms. Results. Thirty‐five had undergone cardiovascular reinterventions. Sixteen had an aortic and three had a mitral valve prosthesis; 117 had a native aortic valve that was bicuspid in 63 and dysfunctional in 45. Ejection fraction was below 50% in 16. On exercise, performance was reduced in 37 and hypertension was induced in 47. Fifty‐eight had elevated blood pressures and further 17 received antihypertensives. The ascending aorta was aneurysmal in 28 and the distal arch in five. The presence of a bicuspid aortic valve was significantly associated with valve regurgitation and ascending aortic ectasia. Fifty‐eight of 121 patients had minimal aortic arch diameters between 46% and 79% of the diaphragmatic aortic diameter, indicating moderate/mild recoarctation. This was associated with elevated blood pressures and use of antihypertensive medication, but not with hypertension in unmedicated patients or with echocardiographic or exercise parameters. Only five patients had normal study findings, were normotensive, and without reinterventions after coarctation repair. Conclusions. Cure by repair of aortic coarctation is rare; heart diseases, aortopathy, and hypertension are common. Morbidity is only weakly associated with mild/moderate recoarctation.     

Cor Triatriatum Sinister with and without Left Ventricular Inflow Obstruction: Visualization of the Entire Supravalvular Membrane by Real‐time Three‐dimensional Echocardiography. Impact on Clinical Management of Individual Patient

We present 4 cases of cor triatriatum in whom the diagnosis was correctly made by 2‐dimensional transthoracic echocardiography, which showed the supravalvular left atrial membrane that divides the left atrium into 2 chambers. The pulmonary veins were connected normally to the proximal left atrial chamber and the left atrial appendage was connected to the distal left atrial chamber. In 1 patient there was evidence of severe pulmonary venous obstruction to the mitral valve by Doppler examination, while in the other three, there was no venous obstruction. Patients were then examined by real‐time 3‐dimensional echocardiography (RT3DE, using ×4 matrix array transducer connected to Sonos 7500 echocardiographic system Phillips, Andover, Mass, USA). This showed the exact morphology of the membrane and led to cancellation of planed surgical intervention in 1 case in which the membrane was only a broad band crossing the left atrial cavity. In addition to delineating the exact morphology of the intracavitary anomaly, this novel echocardiographic imaging modality should be an additive tool to better understand the natural history of these nonobstructive left atrial membranes via longitudinal follow‐up of these patients.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Management of a dissection of matrix P right ventricular‐to‐pulmonary artery conduit by implanting two pre‐stents and a melody valve

Reconstructing the right ventricular outflow tract and pulmonary valve via a bovine‐derived valve conduit such as Matrix‐P‐Xenograft is a common surgical repair technique for pulmonary atresia and ventricular septal defect. After conduit degeneration due to calcification or aneurysmal dilatation, percutaneous transvenous stenting of the right ventricular outflow tract followed by pulmonary valve implantation has become the standard interventional treatment. Applied to stenotic conduits, the method is considered safe and effective. An important but seldom‐reported problem is graft failure related to the formation of a Matrix membrane due to inflammation and fibrosis inside the xenograft, which can cause serious problems when dissection and rupture occur during transcatheter intervention. The torn pseudomembrane may cause the complete obstruction of both pulmonary arteries, resulting in a life‐threatening situation requiring rapid intervention, as in this case presentation. © 2016 Wiley Periodicals, Inc.     

Native Aortic Valve Endocarditis Complicated by Pseudoaneurysm of Mitral‐Aortic Intervalvular Fibrosa

Pseudoaneurysm of mitral‐aortic intervalvular fibrosa (PA‐MAIVF) is a rare complication of native aortic valve endocarditis. This region is a relatively avascular area and prone to infection during endocarditis and subsequent aneurysm formation. The rupture into the pericardial cavity and left atrium or aorta, systemic embolism, myocardial infarction secondary to left coronary compression, and sudden death are the reported complications. Herein, we present a 9‐year‐old boy who was diagnosed with bicuspid aortic valve endocarditis complicated by PA‐MAIVF, cerebral embolism, and hemorrhage. PA‐MAIVF was visualized by both two‐ and three‐dimensional transthoracic echocardiography and ruptured into pericardial space causing a fatal outcome.     

Diagnostic Role of Magnetic Resonance Imaging in Identifying Aortic Arch Anomalies

Purpose. The aim of this article was to assess the role of MRI in the diagnosis and management of a variety of complex aortic arch anomalies. Materials and Methods. Imaging was performed on a 1.5T Philips Gyroscan Intera. We retrospectively reviewed all cardiac MR scans performed from November 2003 to February 2007 at our institute to identify aortic arch anomalies. Magnetic resonance imaging included three‐dimensional (3D) sagittal volume images, cine images, short axis ventricular volumes, phase contrast flow of great vessels, and 3D gadolinium magnetic resonance angiogram. Results. Three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right‐sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, six patients were symptomatic, and four of them underwent a surgical division. Magnetic resonance imaging was used to plan the surgical management. In the other two groups, no patient presented with symptoms of airway or esophageal compression, and the arch abnormalities were noticed during investigation for other associated cardiac disease. However, we noticed a strong correlation with well‐defined subgroups of congenital heart disease. Right‐sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%). Seventy percent of the patients with aberrant right subclavian artery had aortic coarctation, and another case presented a complete aortic arch interruption. Conclusion. Magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies.     

Double Aortic Arch with Aortic Atresia and Left-Sided Type B Interruption

Aortic valve atresia with interruption of the aortic arch is an extremely rare anomaly; only eleven cases of this anomaly have been reported to date. In the absence of additional sources of blood flow to the ascending aorta, aortic valve atresia with interruption of the aortic arch is fatal. We present, to the best of our knowledge, the first case of a live birth with aortic valve atresia and interrupted left aortic arch (type B) without evidence of an aorticopulmonary communication or ductal supply to the native ascending aorta. Instead, blood flow to the native aortic root was derived from a persistent right embryonic dorsal aorta.     

Distal aortopulmonary septal defect,aortic origin of the right pulmonary artery,intact ventricular septum,patent ductus arteriosus and hypoplasia of the aortic isthmus: A newly recognized syndrome

The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and Interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to “steal” from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.     

Self‐repositioning of an embolized patent ductus arteriosus device—a nightmare turned into sweet dreams

A 7‐month‐old boy was admitted for the device closure of symptomatic patent ductus arteriosus (PDA) with moderate pulmonary hypertension. The PDA measured 4.2 mm with adequate ampulla. It was closed with an 8–6 mm Heart R device from pulmonary artery (PA) end. Post extubation, the device embolized to proximal descending thoracic aorta just distal to PDA ampulla. While attempting to snare from the venous side, the device self‐repositioned to PDA. It was stable thereafter and patient was discharged after 2 days. We report a complication, which got self‐corrected. © 2014 Wiley Periodicals, Inc.     

Multiple Coronary Artery Aneurysms of the Right Coronary Artery in Neonate with Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PA/IVS) with right ventricular‐dependent coronaries is a rare congenital cardiac anomaly. It has been associated with wide range of considerable anomalies including sinusoids, fistulae, coronary stenosis, or complete atresia. Coronary artery aneurysms in a neonate with PA/IVS have not been described. We report a case of neonate with pulmonary atresia with intact ventricular septum with giant coronary artery aneurysms.     

Surgical Strategy for Aortic Coarctation Repair Resulting in Physiologic Arm and Leg Blood Pressures

Objective. The goal of aortic coarctation repair is laminar aortic blood flow resulting in a negative or absent arm:leg blood pressure (BP) gradient. Despite satisfactory relief of coarctation, associated arch hypoplasia can result in residual obstruction and postoperative upper body hypertension. Intervention. We devised a surgical strategy to create a tension‐free anastomosis with a diameter as large as both the adjacent proximal and distal aorta using a radically extended end‐to‐end anastomosis via sternotomy and/or thoracotomy. Sternotomy is chosen when there is significant transverse arch hypoplasia defined as a distal transverse arch ≤ diameter of the left carotid artery, presence of a common brachiocephalic trunk, or coexisting intracardiac lesion requiring repair. Thoracotomy is used in all other cases. Results. From 2000 to 2008, 95 consecutive patients were repaired using this approach, 35 with sternotomy and 60 with thoracotomy. At a mean follow‐up of 50 ± 23 months, mean systolic BP was 94 ± 10 mm Hg, and 84% of patients had no residual arm:leg BP gradient. Mean arm:leg BP gradient was not statistically different between groups (?8.5 ± 15 sternotomy and ?7.0 ± 10 mm Hg thoracotomy, P= .7). With Doppler echocardiography, 96% of patients demonstrated normal early diastolic reversal of blood flow in the descending thoracic aorta. Conclusions. For aortic coarctation repair in infancy, a strategy designed to directly address aortic arch hypoplasia results in excellent intermediate‐term results with normal BP, physiologic arm:leg BP relationship, and near normal descending aortic blood flow velocities by Doppler.     

Collateral pulmonary circulation in Fallot's tetralogy

An angiographic study of 231 patients with tetralogy of Fallot (TOF) in both neonatal and adult age groups (0 to 49 years) was in this condition. Cases of pulmonary atresia with ventricular septal defect were excluded. Two types of collateral pulmonary circulation were observed: the most common form is the result of distal intrapulmonary anastomosis between systemic parietal, mammary and bronchial vessels with the pulmonary arteries. This type of collateral circulation represents an evolutive stage of TOF and is directly related to age and to the degree of hypoxia; the other, oa rare and probably congenital form (1.7 p. 100 of cases) observed even in young children, consisting of proximal anastomoses between systemic vessels arising from the aorta (1 to 3 vessels) and the pulmonary arterial tree. The rare forms of TOF with single pulmonary arteries acquired after palliative or congenital anastomoses (1.4 p. 100) have both types of collateral circulation. Preoperative diagnosis of the collateral pulmonary circulation requires opacification of the aorta especially of the descending thoracic segment and its branches, completed in cases of an absent pulmonary artery by wedged pulmonary venous opacification. The haemodynamic effects of this collateral circulation on the pulmonary bed to a variable degree of histological change. This may partially explain some of the poor surgical results of repair of TOF and, in view of recent progress in cardiopulmonary bypass techniques in children, incite to earlier surgical correction of this condition.