Haifa Abdul Latiff

Transcatheter closure of multiple muscular ventricular septal defects using Gianturco coils

A 10-month-old boy underwent operation to close a large secundum atrial septal defect and multiple muscular ventricular septal defects. Closure of the ventricular septal defects was unsuccessful and led to worsening cardiac failure and inability to wean the patient from mechanical ventilation. Transcatheter closure of the ventricular septal defects using Gianturco coils was undertaken. This technique is an effective alternative for closure of multiple muscular ventricular septal defects in infants and small children.

Transcatheter occlusion of native persistent ductus arteriosus using conventional Gianturco coils

Two hundred eleven patients with small- to moderate-sized native patent ductus arteriosus underwent closure using Gianturco coils, employing the transvenous multiple catheter approach. Short-term results showed a high rate of complete occlusion and a potential long-term complication of mild left pulmonary artery stenosis in a small number of patients.

Risk factors for augmentation of the flow of blood to the lungs in pulmonary atresia with intact ventricular septum after radiofrequency valvotomy

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract. The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve. We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups. The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of -1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of -0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle. The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

The value of transesophageal echocardiography in transcatheter closure of atrial septal defects in the oval fossa using the Amplatzer septal occluder

BACKGROUND From January, 1997, as part of an international multicentric trial, we have been closing small-to-moderate atrial septal defects within the oval fossa using the Amplatzer Septal Occluder (ASO, AGA Medical). METHODS All patients with defects within the oval fossa deemed potentially suitable for transcatheter closure were investigated by transesophageal echocardiography with the aim of gaining extra information that might alter the decision to use the device to close the defect. Views were obtained in transverse and longitudinal planes, permitting measurements of the diameter of the defect, and its distance from the atrioventricular valves, coronary sinus, and pulmonary veins. Additionally, we sought to identify multiple defects, and to exclude sinus venosus defects. RESULTS Of 56 patients with left-to-right shunts, 41 (73.2%) were deemed suitable for closure with the Amplatzer Septal Occluder. All underwent the procedure successfully, with no complications. This includes 5 patients with multiple small defects that were sufficiently close to the main defect to be closed with a single device. Only two of these had been detected on the transthoracic study. In the remaining 15 of 56 patients, transcatheter closure was deemed unsuitable. In 9 patients, this was due to the limitation of the size of the device available during the period of study, this representing a relative contraindication. In the remaining 6 (10.7%), transcatheter closure was not performed because multiple defects were too far apart to be closed with a single device in 3 patients, two patients were noted to have a sinus venosus defect, and another was noted to have anomalous connection of the right upper pulmonary vein to the right atrium. Excluding patients contraindicated due to the size of the defect alone, transesophageal echocardiography provided extra information in one-tenth of our patients, which altered the decision regarding management. CONCLUSION Transesophageal echocardiography is indispensable in the evaluation of patients undergoing transcatheter closure of atrial septal defect.

Inverted Left Atrial Appendage Masquerading as a Cardiac Mass

An inverted left atrial appendage is a rare phenomenon post cardiac surgery. The lesion presents as an additional mass in the left atrium, which would trigger unnecessary concerns and frequently, a battery of tests. The lesion can be easily diagnosed using echocardiography. We report a case of inverted left atrial appendage in a patient post repair of common arterial trunk. Echocardiographic pictures and features which help to identify this lesion as well as to differentiate it from other possible left atrial mass are described. This article aimed to improve the awareness of sonographers toward this rare but possible post operative lesion.

An Accessory Mitral Valve Leaflet Causing Left Ventricular Outflow Tract Obstruction and Associated with Severe Aortic Incompetence

This case report describes a 20‐year‐old woman with Turners syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow‐up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane‐like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the “subaortic membrane” had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two‐dimensional and three‐dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction. (Echocardiography 2012;29:E34‐E38)

Persistent valve of systemic venous sinus: a cause of neonatal cyanosis.

Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

Primary Arterial Switch Operation for Late Referral of Transposition of the Great Arteries with Intact Ventricular Septum in the Current Era: Do We Still Need a Rapid Two-Stage Operation?

Objective: We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness. Methods: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age. Results: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (<4 mm [preoperative echo]: n = 13) had significantly longer ventilation time (days; 10.6 ± 4.8 vs 4.8 ± 1.7, P = .0039), and the intensive care unit stay (days) was 14 ± 9.2 versus 7.5 ± 3.5; P = .025, compared with thick LV wall group (≥4.0 mm: n = 13). Conclusions: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.

Right aortic arch with isolation of the left subclavian artery: a rare association with airway obstruction.

We present two interesting cases of isolated left subclavian artery from the pulmonary artery with symptoms of upper airway obstruction. The first patient had tetralogy of Fallot, pulmonary artery sling, bilateral superior caval veins, and left bronchial isomerism, suggesting heterotaxy syndrome. The second patient had a right aortic arch, isolated left subclavian artery, and bilateral arterial ducts. These two cases are interesting because of their rarity and uncommon presentation.

Transcatheter closure of defects within the oval fossa using the Amplatzer Septal Occluder.

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 +/- 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.