Two‐ and Three‐Dimensional Transthoracic Echocardiography in the Assessment of Aortic Arch Vasum Vasi to Pulmonary Artery Fistula

We report an elderly patient presenting with a stroke and also hemolytic anemia secondary to mitral and tricuspid valve repair 3 years earlier, in whom two‐dimensional transesophageal echocardiography (2DTEE) suspected a fistula into the pulmonary artery (APAF) from the posterior wall of the aortic arch. For further assessment, two‐dimensional transthoracic echocardiography (2DTTE) and live/real time three‐dimensional transthoracic echocardiography (3DTTE) were performed. 2DTTE showed findings similar to 2DTEE. Color Doppler guided continuous‐wave Doppler showed continuous flow throughout the cardiac cycle with systolic preponderance across the fistula with a small peak pressure gradient of 22 mmHg. Sequential systematic cropping of the 3D datasets obtained from 3DTTE confirmed that the flow signals originated from the posterior wall of the aortic arch and not the lumen. Thus, 3DTTE served to increase the confidence level that the fistula most likely originated from a vasum vasi in the wall of the aortic arch. To our knowledge, this type of fistula has not been reported previously. Fistulas from the aortic lumen to the pulmonary artery are also rare and so far only 24 cases have been reported in the literature. These are also reviewed by us.     

Intraoperative Transesophageal Echocardiographic Imaging of Intrapulmonary Tunnel Repair for Anomalous Left Coronary Artery Originating from the Pulmonary Artery

Reestablishment of aortic flow to the coronary vasculature is the optimal treatment for anomalous origin of the left coronary artery originating from the pulmonary artery (ALCA). We performed intraoperative transesophageal echocardiography (TEE) in five patients (median age 10 months; range 1.5–36 months) who underwent revascularization of the left coronary artery without coronary mobilization by creation of an aortopulmonary window and intrapulmonary tunnel to the os of the anomalous vessel. Tunnel geometry as well as supravalvar pulmonary course were well imaged. Revascularized left coronary artery antegrade flow from the aorta was documented in all via color and pulse wave Doppler. A peribaffle leak (left-to-right shunt) was noted in 2 of the 5 patients, however its presence did not influence left coronary artery flow or clinical outcome. Intraoperabve TEE is helpful in assessing adequacy of repair and documenting antegrade aortic flow in the left coronary artery after intrapulmonary tunnel repair for ALCA.     

Scanning suprasternal echocardiography.

Scanning suprasternal echocardiography was performed in 280 patients with a variety of cardiac anomalies. By using the special suprasternal transducer on the suprasternal notch, the aortic arch, right pulmonary artery, and left atrium were recorded superoinferiorly. From this reference position various scanning techniques were made to record the main pulmonary artery, pulmonary valve, ascending aorta, aortic valve, and distal aortic arch, wherever possible. These scans made it possible to assess (a) the integrity and size of right pulmonary artery and main pulmonary artery in pulmonary atresia, stenosis of origin of right pulmonary artery, pulmonary artery banding, kinked Waterston anastomosis, and aneurysm of pulmonary artery; (b) relative positions of aortic valve and pulmonary valve in malposition complexes; (c) the position and size of the aortic arch in tetralogy of Fallot and aortic coarctation. Measurements of the left atrium made by suprasternal echocardiography were consistently larger than those made by praecordial echocardiography. Changes in relative sizes of aortic arch, right pulmonary artery, main pulmonary artery, and left atrium were also documented in the various cardiac anomalies. The atrial baffle after Mustard repair for d-transposition of the great arteries and the atrial membrane in cor triatriatum were also demonstrated. The introduction of scanning techniques has thus widened the scope of suprasternal echocardiography.     

A Serendipitous Identification of a Lead Extraction Complication

A 57‐year‐old male with a dual chamber pacemaker and symptomatic, persistent atrial fibrillation (AF) accompanying a febrile illness presented for elective transesophageal echocardiography (TEE)‐guided cardioversion. The patient was found to have a large 2.5 cm × 2.3 cm, mobile mass attached to the right atrial lead. Following device and lead extraction, he developed progressive shortness of breath which was attributed to his underlying arrhythmia. One month later, AF ablation was pursued and preablation TEE revealed a dilated main pulmonary artery with a 1.8 cm × 1.6 cm mass in the distal left pulmonary artery, which was corroborated by a 1.4 cm × 2.5 cm filling defect in the descending left pulmonary artery on magnetic resonance angiography (MRA). To our knowledge, this is the first case report to document the distal migration of vegetation material into the pulmonary artery with serial TEE and highlights the risk of pulmonary embolism (PE) in patients with large endocardial lead vegetations undergoing transvenous lead extraction.     

Saccular aneurysm of the transverse thoracic aorta detected by transesophageal echocardiography

A patient with a saccular aneurysm of the thoracic aortic arch presented with severe right ventricular failure due to pulmonary artery compression. Contradictory data were derived from computed tomography, pulmonary isotope perfusion scan and cardiac catheterization. Transesophageal echocardiography revealed a saccular aneurysm which compressed the main pulmonary artery and gave access to a fistulous connection to the left pulmonary artery. Surgery confirmed these findings.     

Two‐ and Three‐Dimensional Transthoracic Echocardiography in the Assessment of Acquired Ascending Aortic Aneurysm to Pulmonary Artery Fistula

Aorta to pulmonary artery fistula is an uncommon and potentially fatal condition. This case is of a 48‐year‐old Caucasian male with congestive heart failure and multiple aortic valve replacement surgeries who presented with an acquired ascending aortic aneurysm to pulmonary artery fistula diagnosed using two‐dimensional transthoracic echocardiography via nonstandard imaging windows. Three‐dimensional transthoracic echocardiography using live/real time three‐dimensional color Doppler was used to assess the size of the opening of the fistula, providing additional value. This patient was surgically managed and is doing well 8 months postoperation.     

Spontaneous Closure of a Symptomatic Coronary Artery Fistula Just within a Few Days of Newborn Period

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14‐day‐old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small‐sized fistulas opening into the right‐sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.     

Echocardiographic Diagnosis and Transcatheter Occlusion of Pulmonary Arteriovenous Fistula in Cyanotic Newborn

Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in newborn. A 12‐day‐old male newborn (2.8 kg) was referred to our hospital with the complaints of cyanosis and respiratory distress. On two‐dimensional echocardiography, the right pulmonary artery (PA) appeared larger than left PA and the left atrium, left ventricle were dilated. The right heart chambers were in normal limits. A color flow Doppler echocardiogram revealed a turbulent flow due to a PAVM originating from medium branch of right PA, and continuous wave Doppler showed continuous flow pattern. Agitated saline injection resulted in the delayed appearance of the contrast in the left‐side chambers three to four heart cycles after appearance in the right‐side chambers; the study was considered positive and indicative of an intrapulmonary shunt. Selective angiography of the right PA confirmed the diagnosis of a large solitary PAVM in the right middle lobe with a feeding artery. Amplatzer vascular plug I, which is designed to close abnormal vascular structures, was chosen to close the PAVM. The deployment of device performed safely and the oxygen saturation of baby increased to 95% immediately after deployment. Heart failure and respiratory distress also resolved after the procedure.     

Rupture of an aortic dissection into the right atrium in a patient with a previous aortic valve replacement: a case report.

We report the case of a 73-year-old man with a history of previous aortic valve replacement in 1990 and rupture of an aortic dissection into the right atrium. The patient was admitted to the emergency room because of chest pain, stopped not long after. The electrocardiogram did not show any signs of ischemia and myocardial enzymes were not increased. Transthoracic echocardiography revealed aortic root dilation (maximum diameter 60 mm) extended to the aortic arch, and the presence of a flow from the ascending aorta to the right atrium (evocative of a fistula between the two chambers). The aortic valvular prosthesis function was good. Transesophageal echocardiography confirmed an aorta-right atrium fistula. Cardiac catheterization did not show any luminal obstructions in the coronary arteries; there was a small shunt from the aorta to the right atrium. The ascending aorta and the aortic root were replaced with a Dacron graft. Right and left sinuses were reimplanted to the graft. The fistula was repaired with 4-0 pledgeted Prolene sutures. The surgeon's diagnosis was "type A aortic dissection in a patient with an ascending aorta aneurysm and an old ascending aorta-right atrium fistula".     

Acquired aortopulmonary fistula

An acquired aortopulmonary artery fistula is rare. We describe a case with an aortic arch aneurysm communicating with the main pulmonary artery. The diagnosis was made on the basis of transthoracic echocardiography and confirmed by transesophageal echocardiography. A post-mortem examination revealed the complete anatomy of the aneurysm and the aortopulmonary communication.     

Mycotic aneurysm of the root of left subclavian artery associated with aortic coarctation discovered by echocardiography

A 16‐year‐old girl was admitted with a 7‐month history of recurrent fever, weight loss of 5 kg, and general poor health status. Blood culture was positive with Streptococcus viridans. Transthoracic echocardiography established an aneurysm of the root of left subclavian artery, associated with an aortic coarctation just before the origin of the left subclavian artery with a velocity of 4.8 cm/s. Three‐dimensional echocardiography demonstrated the entire inner structure of the aneurysm. Computed tomography reconstruction confirmed the presence of aortic coarctation and mycotic aneurysm, it also revealed an aberrant right subclavian artery.     

Rupture of a Noncoronary Sinus of Valsalva Aneurysm into the Left Atrium: A Rare Cause of Acute Pulmonary Edema

A sinus of Valsalva aneurysm is a dilatation of the aortic wall caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve. It has an incidence of <0.1%. The most common cause of a sinus of Valsalva aneurysms is congenital, although they may also be acquired. The most common complication is rupture into the right atrium or ventricle, with rupture into the left chambers occurring very rarely. We present a 40‐year‐old man admitted to the hospital with an acute onset of respiratory distress and pleuritic chest pain. Transthoracic echocardiography followed by transesophageal echocardiography showed rupture of a noncoronary aneurysm of Valsalva sinus into the left atrium. The jet from the fistula caused retrograde flow into the pulmonary veins.     

Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery

Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left‐sided brachiocephalic artery in the setting of a right‐sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three‐vessel tracheal view.     

冠状动脉起源于肺动脉的超声诊断研究

目的：冠状动脉起源于肺动脉是一种罕见的先天性畸形，以左主干爱累多见，患者多夭折于婴幼儿期，存活至童年乃至成年仍有猝死的可能。早期诊治尤为重要。本文探讨超声心动图检出该病的可行性。方法：分析５例左主干起源于肺动脉病例的二维超声心动图（２ＤＥ）和彩色多普勒血流显像（ＣＤＦＩ）的发现。其诊断经心血管造影及手术证实。结果：５例均有左室增大，右冠状动脉增宽但起源正常，左主干增宽与主动脉左窦不连接，可探及其在肺动脉干后部的开口；ＣＤＦＩ检出了肺动脉中部的双期连续性血流，其中４例检出了室间隔右缘与之伴行的左右冠状动脉间的交通支。结论：该病具特征性的２ＤＥ与ＣＤＦＩ改变，采用综合超声技术可做出诊断或提示性诊断。     

Three‐Dimensional Transthoracic Echocardiography in Identification of Aorto‐Right Atrial Fistula and Aorto‐Right Ventricular Fistulas

We report the case of a 72‐year‐old woman who developed new onset right ventricular failure after redo aortic valve replacement. The diagnosis of left to right shunt was initially made using two‐dimensional transthoracic echocardiography (2DTTE) and 2D transesophageal echo with color Doppler (TEE). Definite diagnosis of aorto‐right atrial and aorto‐right ventricular fistula was made using thee‐dimensional transthoracic echocardiography (3DTTE) with color flow Doppler imaging. Early recognition and diagnosis of this rare surgical complication is imperative for prompt surgical repair of this lethal defect. 3DTTE should be utilized in cases of new onset heart failure with unclear etiology to diagnose unusual causes of this potentially fatal condition. (Echocardiography 2010;27:E105‐E108)     

Fistula between the right pulmonary artery and the left atrium coexisting with a secundum‐type atrial septal defect: An unusual case of cyanosis in a girl

A fistula between the pulmonary artery (PA) and the left atrium (LA) is a rare congenital heart disease that usually presents with cyanosis, clubbing, and dyspnea, as well as the signs and symptoms of a right‐to‐left shunt. Herein, we report a 16‐year‐old girl with a fistula between the right PA and the LA. This type of fistula could lead to systemic desaturation. This patient also had an atrial septal defect of the secundum type and has been followed up without treatment. The clinical manifestations and treatment of fistulas located between the PA and LA are also reviewed in this report.     

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.     

Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left‐sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).     

A self-resolving,post-traumatic aortopulmonary shunt

A 34-year-old Hispanic man sustained a stab wound to his chest complicated with hemopericardium and pericardial tamponade. He underwent emergent clamshell thoracotomy as well as repair to the pulmonary artery. A transthoracic echocardiogram showed no evidence of intracardiac shunt. Two months later, a new murmur was noted, with a transthoracic echocardiogram revealing high-velocity flow between the left coronary sinus and the main pulmonary artery, with which a coronary computed tomography angiogram concurred. A transesophageal echocardiogram was performed which revealed an aortopulmonic fistula from the left coronary sinus of Valsalva, approximately 1cm anterior to the ostium of the left main coronary artery, to the main pulmonary artery just distal to the pulmonic valve. Pulmonary insufficiency was minimal. The main pulmonary artery was dilated, measuring 3.2 cm by coronary computed tomography angiogram. Right ventricular systolic function was normal. Right and left heart catheterizations were performed to further assess hemodynamics and coronary anatomy; pulmonary artery pressures were 16/8 mm Hg. Aortopulmonary fistula was seen on aortogram. Surgery was deferred in view of lack of symptoms and uncertainty in its natural history in the setting of traumatic etiology. A repeat transthoracic echocardiogram at six-month follow-up showed spontaneous closure of the fistula.