Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.

Check for updatesmitral stenosis, cyanotic CHDs with pulmonary oligemia, stenotic bioprosthetic valves, congenital tricuspid and mitral stenosis, truncal valve stenosis, subvalvar pulmonary stenosis, supravalvar pulmonary stenosis (congenital membranous or postoperative), stenosis of the aorta (Leriche syndrome, atherosclerotic and Takayasu's arteritis), baffle obstruction following Mustard or Senning procedure (both systemic and pulmonary venous obstructions), superior and inferior vena caval obstructions, pulmonary vein stenosis, pulmonary veno-occlusive disease, vertical vein stenosis in total anomalous pulmonary venous connection, pulmonary venous obstruction following repair of total anomalous pulmonary venous connection, specially designed pulmonary artery bands, cor triatriatum, cor triatriatum dexter, and coronary artery stenotic lesions that develop after Kawasaki disease; 2) Stents to enlarge stenotic lesions of branch pulmonary arteries, systemic veins, systemic and pulmonary venous pathways after Mustard procedure, aorta, right ventricular outflow conduits, pulmonary veins and native right ventricular outflow tract or to keep the ductus arteriosus open in patients with pulmonary atresia and hypoplastic left heart syndrome and maintaining patency of stenosed aorto-pulmonary collateral vessels, surgically created but obstructed shunts or acutely thrombosed shunts as well as covered stents; 3) Trans-catheter occlusion of cardiac defects comprising of atrial septal defect,

Other contributions are: Review of evaluation of left ventricular function by echocardiography [26,27], echocardiographic diagnosis of tricuspid atresia [28], utility of contrast echocardiography in the assessment of hypoxemia after open heart surgery [29], quantifying left ventricular muscle mass by m-mode echocardiograms in children [30], racial and sexual differences in echo measurements in children [31], heart size and function in children with sickle cell disease [32], and after-

Check for updatesphysiologically advantageous VSDs occurs as often as in isolated VSDs. The mechanisms of closure is also similar to that seen in isolated VSDs. VSD closures were documented to take place in utero, during infancy, childhood, and adolescence and continue through adulthood. It was concluded that there is a great natural tendency for spontaneous closure of VSDs whether it be an isolated defect or is a component of a complex heart defect [10][11][12][13][14][15][16].Spontaneous closure of VSD in children with tricuspid atresia and normally related great arteries (Type I) produces decreased pulmonary blood flow and requires surgical intervention earlier than otherwise needed. We recommended that a Blalock-Taussig (BT) shunt (preferably on the left side) is carried out instead of a Glenn anastomosis (the concept of bidirectional Glenn has not yet been crystallized as of that time) because VSD closure following Glenn will result in no blood flow into the left pulmonary artery (Figure 3). If further palliation is required prior to Fontan procedure, a BT shunt on the right side, Glenn anastomosis, or enlargement of the VSD may be entertained. In subjects with tricuspid atresia and transposition of the great arteries (Type II), VSD closure produces left ventricular outflow obstruction. Therefore, it is highly important that the size of the VSD is appraised before planning for Fontan. If the VSD is small, enlarging the VSD surgically or bypassing the VSD and the right ventricle via a pulmonary artery-to-ascending aorta anastomosis (Damus-Kaye-Stansel) or by a left ventricle-descending aorta conduit (Figure 4) should be considered [10][11][12][13][14]. In addition to tricuspid atresia and double outlet right

Check for updatesdevice. A methodical assessment of all three cohorts of the buttoned device suggested progressive decrease in complications rates, including unbuttoning and better implantation feasibility while preserving the effective occlusion rates with consecutive re-designed versions of the buttoned device [17,18,23]. The author's experience with Amplatzer Septal Occluder (ASO) was then reviewed with the conclusion that ASD occlusion with ASO is feasible, safe and effective in most types of ostium secundum ASD cases with occasional complications [9,10,24]. Initially, the ASO device characteristics were described followed by presentation of indications for device closure, the technique of device implantation, and results (Figure 4).The author and his associates performed the closure of more than 100 ostium secundum ASDs with the ASO as of 2011. The initial series of patients was presented in a poster format [24]. Sixty-five patients were taken to the catheterization laboratory with the intent to occlude their ASDs with the ASO during 2003 to 2011. Devices were not implanted in 4 (6%) patients because of flail or inadequate septal rims. In the remaining 61 patients, transcatheter occlusions were performed with the device. The ages of the patients varied between 3.1 and 17 years (mean 7.9 years). The weights of the patients ranged between 11.8 and 69 kg with a mean of 29.6 kg. Twenty-four were boys and 42 were girls. The Qp: Qs was 0.9 to 4.5 with a mean of 1.8. The ASD diameter measured by TEE varied between 5.6 and 29.5 mm with a mean of 14 mm. The ASD diameter measured by