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We present a case of dilated main pulmonary artery diameter with accompanying cardiac anomalies and postnatal follow-up. The abnormalities in the left side of the heart concerned the left superior vena cava (LSVC), decreased left ventricular contractility SF LV (shortening fraction), and narrowing/accelerating flows through the aortic valve and aortic arch isthmus. In our case, coarctation of the aorta was suspected both pre- and postnatally due to the dominant picture of the accelerated flow velocities, but the patient did not require neonatal surgery. Based on a retrospective review of the case, we tried to explain our prenatal and early postnatal findings. After the exclusion of the structural congenital heart defects as a base of the dilatation of the main pulmonary artery, we should consider other pathophysiological mechanisms presented in the following report.

Congenital coronary artery anomalies are rare but well-described causes of chest pain and, in some cases, link to sudden cardiac death. With the spread of advanced imaging techniques, the number of incidental findings is staggering, but little information has been given in order to rule out potential malignant cases in symptomatic adult patients. Here, we describe a case of an anomalous course of the coronary artery with an acute (inter-arterial course between a dilated ascending aorta and a dilated pulmonary artery, and how we could manage this patient in our clinical practice.

The extrinsic compression of the left main coronary artery secondary to pulmonary artery trunk dilatation is a relatively rare syndrome. [1,2] It is mostly associated with congenital acyanotic heart disease, idiopathic pulmonary arterial dilatation or primary pulmonary hypertension. [3,4] Most cases of pulmonary artery hypertension with no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is delayed.We describe a patient with Ostium secundum atrial septal defect with severe pulmonary hypertension who presented with clinical angina ,diagnosed to have left main coronary artery compression by CT angiography ,underwent successful percutaneous coronary intervention with good symptomatic relief.

A 39-year-old G3P3 female presented with abrupt onset dyspnoea of one month duration. She was markedly symptomatic when lying supine and resorted to prone sleeping. Chest X-ray reported as cardiomegaly. Transthoracic echocardiography was unremarkable twice. Computed tomography chest showed a dilated pulmonary artery. Transesophageal echocardiography identified a 12-mm ostium secundum atrial septal defect with mild pulmonary hypertension. The defect was closed with a cocoon device and rendered her symptom free. This case highlights the importance of timely organization of transesophageal echocardiography when transthoracic echocardiography is negative. It also illustrates marked dyspnoea could be a presentation of undiagnosed atrial septal defect with mild pulmonary hypertension.

Background Idiopathic dilatation of the pulmonary artery (IDPA) is a rare condition in which the pulmonary artery dilates without an obvious cause. Pulmonary artery replacement is indicated in severe cases to prevent serious complications. Case presentation A 59-year-old man was diagnosed with an IDPA of 64 mm and Kommerell’s diverticulum (aortic aneurysm located at the aberrant left subclavian artery). A computed tomography scan revealed slight compression of the aneurysm to the trachea, although not interfering with airway management. The surgical approach was a median sternotomy, and cardiopulmonary bypass was established through aortic and bicaval cannulations. The perioperative course was uneventful. Conclusions To prevent injury to the dilated pulmonary artery, a strategy for cardiopulmonary bypass and a surgical approach should be discussed beforehand. As dilatation of the pulmonary artery is often complicated by anatomic abnormalities, preoperative evaluation should be aimed at appropriate assessments using imaging modalities.

Airway obstruction from an enlarged pulmonary artery (PA) is not a common occurrence. We present a rare case of respiratory failure secondary to right bronchus obstruction from a dilated right PA. A 54-year-old male with a known history of pulmonary hypertension (PH) and obstructive sleep apnea (OSA) presented with worsening dyspnea. He was found to have collapse of his right middle and lower lobes. Intubation was required for respiratory failure. To our knowledge, this is the first case to be reported in the literature where PH caused PA dilatation to such a degree as to cause bronchial obstruction and subsequent lobar collapse.

A 51-year-old man presented with non-ST-elevation myocardial infarction due to extrinsic compression of the left main coronary artery (LMCA) caused by a dilated pulmonary artery (PA) with secondary pulmonary hypertension and was successfully treated with surgical intervention including pulmonary angioplasty, atrial septal defect correction, and thromboendarterectomy. Imaging modalities were extremely useful in making the diagnosis and providing follow-up of LMCA compression syndrome in this case. During the follow-up, a sufficient hemodynamic improvement was obtained, without exacerbation of the PA dilatation, resulting in the absence of compression of the LMCA.

Of the associated cardiac defects with absent pulmonary valve, the combination of tricuspid atresia, ventricular septal defect, and aneurysmal dilatation of the pulmonary arteries is very rare. We report the case of a low-birth-weight girl (2,282 g) with this anomaly, which was prenatally diagnosed. Fontan completion was successfully achieved at 16 months of age, following staged palliative procedures including banding of the main pulmonary trunk and plication of the aneurysmal dilated central pulmonary artery.

Pulmonary hypertension is the commonest condition leading to dilated pulmonary artery. We describe three different types of compression of adjacent anatomical structures by dilated pulmonary arteries. We included involvement of the left main coronary artery, left recurrent laryngeal nerve and tracheobronchial tree. Compression of these structures can cause major complications such as myocardial ischemia, hoarseness and major airway stenosis. We present a case for each scenario and review the literature for each of these complications, focusing on patients’ characteristics and contemporary management.

A neonate developed severe dyspnea following an arterial switch operation due to compression of the trachea and left main bronchus by a dilated pulmonary artery. This was relieved by a second procedure in which the dilated pulmonary artery was excised and reconstructed with bovine pericardium.

Cardiovocal syndrome, also known as Ortner's Syndrome, is a rare cause of unilateral vocal fold paralysis (UVFP) associated with cardiac failure. Typically, UVFP is left sided and related to compression or stretching of the left recurrent laryngeal nerve. The original clinical description relates UVFP to left atrium enlargement as heart failure progresses in the context of mitral stenosis. However, other cardiac abnormalities have since been described. Here we present the case of a 7-week old infant who initially came to the attention of pediatric cardiology due to failure to thrive in the context of a grade 4 systolic murmur. Laryngoscopy revealed UVFP on the left side. Echocardiography and MRI scanning confirmed atrial and ventricular septal defects and pulmonary artery dilatation as the likely cause of left recurrent laryngeal dysfunction. Keywords: Cardiovocal syndrome, Dilated, Pulmonary artery, Ortner's syndrome, Left recurrent laryngeal nerve

Patients with systemic sclerosis (SSc) are routinely investigated with high-resolution computed tomography (HRCT) chest for early detection and accurate characterization of complicating interstitial lung diseases. Though the primary aim of HRCT is to delineate the burden of pulmonary involvement and to characterize the nature of fibrosis to potentially help guide management, it provides an opportunity to evaluate extrapulmonary manifestations, particularly the dilated pulmonary artery, esophageal dilatation, and pericardial abnormalities which have their own clinical significance. The aim of this article is to discuss the significance of various pulmonary and extrapulmonary abnormalities that may be identified on HRCT chest of SSc patients.

A 68-year-old woman with long-standing severe pulmonary arterial hypertension was referred for coronary catheterization for investigation of worsening exertional dyspnea associated with angina. Coronary angiography revealed critical ostial left main coronary artery (LMCA) stenosis. Given the

We thank Dr. Montero-Cabezas and colleagues for the suggestions aimed to optimize the evaluation of left main coronary artery (LMCA) stenosis severity due to extrinsic compression from dilated pulmonary artery (PA) in patients with pulmonary arterial hypertension (PAH). They propose to use

We performed anterior translocation of the pulmonary artery (Le-Compte's maneuver) in a patient with an atrial septal defect with long standing complete collapse of the left lung due to extrinsic compression of the left bronchus by a dilated pulmonary artery. Following the closure of the septal defect and anterior translocation of the pulmonary artery, there was immediate relief of airway obstruction followed by reexpansion of the collapsed lung. (J Card Surg 2011;26:111-113)

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare anomaly presenting with enlargement of the pulmonary artery in the absence of an identifiable cause. The natural history of this silent but potentially life-threatening disease can be unpredictable. We report a case of IDPA in an 80-year-old female with a massively dilated pulmonary artery.

Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.

Tracheobronchial compression is an important cause of respiratory distress in children, requiring precise diagnosis and early surgical management. Common causes of the compression are either intrinsic or extrinsic, the latter being usually due to vascular rings. We report a 10 weeks old boy in whom a dilated pulmonary artery due a large patent ductus arteriosus was the cause for extrinsic compression.

Ortner syndrome or cardiovocal syndrome refers to hoarseness of voice due to recurrent laryngeal nerve paralysis secondary to cardiovascular disease. We present three cases of Ortner syndrome in infants with congenital heart disease. All the three cases had moderate to severe pulmonary hypertension with moderately dilated pulmonary artery. We believe that the dilated pulmonary artery caused compression of the left recurrent laryngeal nerve resulting in hoarseness of voice.

We describe an 11-year-old girl with tetralogy of Fallot and absent pulmonary valve, who on selective coronary angiography was found to have extrinsic compression of the left main coronary artery by the aneurysmally dilated pulmonary artery. This abnormality has not been reported previously. Cathet. Cardiovasc. Intervent. 46:438–440, 1999. © 1999 Wiley-Liss, Inc.

This report describes a case of unroofed coronary sinus and persistent left superior vena cava discovered during an echocardiographic investigation for dilated pulmonary artery. An unroofed coronary sinus is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava. The latter is a usual cause of a dilated coronary sinus. The detection of a dilated coronary sinus should therefore prompt the search for abnormal coronary sinus drainage and other cardiac abnormalities. The treatment of unroofed coronary sinus and persistent left superior vena cava is undertaken only after assessing the pre- and post-treatment haemodynamics of all co-existing abnormalities.

Two infants with tetralogy of Fallot with absent pulmonary valve were studied with electrocardiographically gated magnetic resonance imaging using a 1.5 T imaging system. In each case, imaging was performed successfully. The magnetic resonance imaging clearly demonstrated a rudimentary pulmonary valve, an aneurysmal pulmonary artery, and compression of the mainstem bronchi. Cine MRI revealed phasic compression of the bronchi by an aneurysmally dilated pulmonary artery. Magnetic resonance imaging produced high quality images and provided all of the anatomic information required for preoperative evaluation.

Ventricular and arterial pressure tracings, electrocardiograms and phonocardiograms were studied in 4 normal subjects and 52 patients with atrial septal defect. After excluding cases in which complicating factors were involved, 10 cases of secundum type and 3 cases of primum type were studied in detail. Contrary to previously held beliefs, right ventricular systole is not longer than left ventricular systole in these patients. Therefore, pulmonary valve closure still occurs very close to aortic valve closure, as in normal subjects. However, the incisuras of the large arteries are more widely separated than in normal subjects. The explanation for the wide splitting of the second heart sound is based on recent work that attributes the sound components to vibrations of the blood, valves and walls of the large arteries at the time of the aortic and pulmonary arterial rebounds. Patients with atrial septal defect have a dilated pulmonary artery. This causes a slower reaction of the wall and a later occurrence of both the pulmonary incisura and the pulmonary component of the second heart sound.

1. 1. The factors responsible for normal and abnormal splitting of the second heart sound are reviewed. 2. 2. In 9 patients, abnormal splitting which could not be explained by these factors was present. 3. 3. Marked dilatation of the main pulmonary artery was found in all these patients, suggesting that lack of elastic recoil accounted for the delayed closure of the pulmonary valve. 4. 4. It is suggested that elastic recoil of the pulmonary artery is a hitherto undescribed factor in the mechanism of closure of the pulmonary valve.

1. 1. Two cases of syphilitic aortic insufficiency associated with a large interatrial septal defect are presented. 2. 2. The diagnosis of such a combination of lesions may be suspected before death when the clinical syndrome of syphilitic aortic insufficiency is associated with electrocardiographic evidence of right axis deviation and possibly auricular fibrillation, with roentgenographic findings of a distinctly dilated pulmonary artery.

Chest radiographs obtained at birth in two neonates with absent pulmonary valve and tetralogy of Fallot demonstrated asymmetrical lung aeration. This finding was attributed to delay in resorption of fetal lung fluid. It is postulated that in the initial hours of life, the dilated pulmonary artery compressed the bronchus and delayed egress of fetal lung fluid. Over a 24-hour interval, the fluid was resorbed, resulting in the more typical pattern of hyperinflated lung and markedly dilated pulmonary artery. These cases are presumably the first of their kind to be reported.