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Our objective was to assess the immediate and short-term results of stent implantation to relieve pulmonary artery stenosis (PAS). Thirty-seven patients underwent an attempt at stent implantatlon at a medlan age of 7.0 years (range, 0.G31.4 years) and a median weight of 20.5 kg (range, 7.4-85 kg). Twenty-two patients had previous tetralogy of Fallot repair. A total of 55 stents were Implanted successfully in 36 patlents. The peak systolic gradient across the stenotic segment decreased from a mean of 43 2 20.4 mmHg prestent to 13 f 13.9 mmHg (P a:: 0.001) poststent. The diameter of the narrowest segment Increased from a mean of 4.8 f 1.6 mm to 10.5 f 2.6 mm (P ,:I 0,001). The right ventrlcular-to-aortic mean systolic pressure ratio decreased from 0.74 2 0.2 to 0.52 ? 0.1 9 (P c: 0.001). Complications included balloon rupture prior to full stent expansion in 4 patients (in 2 patients the stent was positioned in the superior vena cava, and in 2 in the inferior vena cava), distal migration of a stent which was successfully retrieved at surgery 1 mo later in 1 patient, and tethering of the stent to the balloon requiring surgical removal in 1 patient. One patient died several hours after stent placement. Sixteen patients underwent repeat catheterization at a mean follow-up interval 010.9 * 0.5years (range, 0.2-2.0years). The mean gradient across the stent for these 16 patients was 26.7 f 19.8 mmHg, and there was no change in the mean diameter (9.4 2 3.2 mm). Two patients developed stenosis related to neointimal proliferation at the stent site which was redilated successfully. In conclusion, stent irnplantation is generally safe and effective in relleving PAS. c 1996 WIIO~-LI~~. Inc

We reviewed a total of 169 deaths among 969 infants referred from various health institutions in the Asir Region to the neonatal intensive care unit of Asir Central Hospital, Abha, Saudi Arabia, during a four-year period (January 1992 to December 1995). The results showed that the major causes of neonatal mortality in the region were low birth weight (LBW) (45%), congenital malformations (CM) (30.8%), infection (13.6%), and birth asphyxia (7.7%). The majority of the LBW infants died from respiratory insufficiency, sepsis, and necrotizing enterocolitis. The alimentary, central nervous and cardiovascular systems were the parts most commonly involved in CM, with cardiac defect accounting for the highest fatality rate due to lack of facilities for cardiac surgery in the region, and delay in effecting a transfer. Klebsiella, Serratia and E. coli are the predominant organisms causing neonatal sepsis, in contrast with the group B streptococcus dominating in America and the United Kingdom. Generally, factors causing neonatal deaths in the Asir region are similar to those reported in other tertiary hospitals in Saudi Arabia, and tally closely with observations in America and Europe, where the standard of health care is excellent. Nevertheless, the study indicates the need for upgrading of facilities, as well as for more trained personnel for obstetric and neonatal care in Asir region. It may be necessary to consider the need for the establishment of a regional cardiac surgery center in the region.

The aim of this study was to assess the frequency of various forms of congenital heart disease (CHD) among affected children in the Southwestern region of Saudi Arabia.During the study period (July 1994 to June 1996), 608 children were referred to Asir Central Hospital as having CHD. All the children were evaluated by a pediatric cardiologist and had electrocardiogram and echocardiogram.Of the 608 patients, only 335, comprising 162 males and 173 females, had CHD. The male to female ratio was 0.9:1. The frequency of various forms of CHD was as follows: ventricular septal defect (VSD) 32.5%; patent ductus arteriosus 15.8%; atrial septal defect 10.4%; pulmonary stenosis 10.1%; atrioventricular septal defect and mitral valve prolapse, 3.6% each; aortic coarctation/interruption 3.3%; obstructive aortic valve lesions 2.7%; tetralogy of Fallot 4.5%; common ventricle 2.7%; pulmonary atresia (PA) with VSD 1.8%; D-transposition of the great arteries 1.5%; Ebstein anomaly 1.5%; and isolated PA 1.2%. Other lesions were extremely rare.The distribution in this study is similar to that reported in previous studies from other parts of the world, except for the lower incidence of obstructive aortic valve lesions.

A six-year-old boy presented to the Cardiology clinic with history of mild cyanosis and dyspnea on exertion from age 1. He had a to-and-fro murmur at the middle left sternal border. Chest examination was normal but chest x-ray showed a small left lung. Echocardiography established the diagnosis of tetralogy of Fallot (TOF) and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. There was severe dilatation of the main and right pulmonary arteries. The left pulmonary artery (LPA) could not be seen. Angiography failed to show a LPA. This case of an absent LPA associated with absent pulmonary valve syndrome is discussed and the literature is reviewed.

A 3-year-old child with a patent arterial duct underwent percutaneous transcatheter occlusion using Rashkind's "double umbrella" technique. The procedure, using a 17 mm device, was uncomplicated. An echocardiogram done 6 hr later showed a mobile 5 x 3 mm thrombus on the pulmonary aspect of the device. The thrombus resolved after 24 hr of intravenous heparin.

SummaryA seven-year-old patient with severe valvar pulmonary stenosis developed a mycotic aneurysm of the pulmonary trunk, which was followed closely with echocardiography. The aneurysm increased rapidly in size over a period of three weeks and required surgical removal.

We have now achieved transcatheter occlusion of the patent arterial duct as a day-case procedure in 26 patients. In all but two, who were judged not to be appropriate for the device, implantation was successful. Most of the patients arrived in the hospital at 8:00 a.m. and had to wait for a mean time of 154 minutes until they were taken for cardiac catheterization. The procedure, performed under sedation in 25 patients and under general anesthesia in two, took a mean of 91 minutes. The children were then monitored for two hours in the recovery room. The interval From the time of arrival in the Unit until waking was a mean of 196 minutes. Then, from the time of waking, a mean of 123 minutes elapsed prior to discharge. Admission of the patient was necessary in three instances (11.1%). Two patients vomited and remained too drowsy for safe discharge. A third patient had either occlusion or thrombosis of the femoral artery and received an infusion of heparin which necessitated admission overnight. All three patients were discharged the following morning. Patients from outside Riyadh stayed overnight in Riyadh before they went home. One patient complained of non-cardiac chest pains a few hours after discharge and was subsequently sent home. Total occlusion, as judged by mean follow-up of 13 months (5–26 months) using color flow Doppler was achieved in 19 of the 24 patients (79!) in whom devices were inserted. All patients are well and there are no complaints concerning the procedure done on a day-case basis. We conclude that the day-case protocol for transcatheter occlusion of the patent arterial duct is a safe and cost-effective option for selected patients.

SummaryA rare association of ‘absent pulmonary valve’ syndrome, midmuscular ventricular septal defect and aneurysmatic dilation of the ascending aorta without pathology of the aortic valve is described in a 10-month-old infant. The diagnosis was established by echocardiography and angiography.

It has previously been reported that in adult patients with sickle-cell anemia the serum phosphate value and the maximum tubular reabsorption of phosphate per liter of glomerular filtrate (TmP/GFR) were significantly higher than in normal controls. This does not appear to have been studied in children with sickle cell anemia (young sicklers) and this prompted us to assess renal phosphate reabsorption in this group of patients. We looked at serum phosphate level and calculated renal phosphate reabsorption (TP/GFR) in children taking random urine and blood samples at the same time and using the formula TP/GFR = Sp – Up × SCr: UCr, in 30 young sicklers all of whom had normal renal function (mean age 7.3 years) and 40 normal matching controls (mean age 6.5 years). The mean serum phosphate value in young sicklers was significantly lower than in controls (4.3 against 5.3 mg/dl) while the mean value of TP/GFR was 4.09 ± 0.74 mg/dl in young sicklers compared to 4.65 ± 0.75 mg/dl in the control group (p = 0.0026). Therefore, the TP/GFR in young sicklers was also significantly lower (p = 0.0026) than in the control group. This may be explained by the high serum level of parathyroid hormone reported previously in patients with sickle cell anemia which is expected to lower phosphate reabsorption (TmP/GFR and TP/GFR are identical in children). The lower serum phosphate value and TP/GFR in younger sicklers seems to be in contrast with the relatively high serum phosphate value and TP/GFR previously reported in adults with sickle cell anemia.

We studied the alpha- and beta-adrenoceptor activity and catecholamine and cAMP levels in 112 children and infants admitted to the hospital for diagnostic or interventional catheterization of tetralogy of Fallot, ventricular septal defects with or without hypertension, pulmonary stenosis, coarctation of the aorta, and various complex cyanotic congenital cardiac diseases and compared them with 14 children undergoing transcatheter occlusion of patent ductus arteriosus with insignificant left-to-right-shunts. The mean total platelet alpha-adrenoceptor density of the study population was elevated by 73%. Both the increases in acyanotic (p < 0.05) and cyanotic (p < 0.005) patients as well as the difference between the two groups (p < 0.01) were significant. Based on the congenital disease classification, the elevation in receptor density was also significant in all groups of patients, except coarctation of the aorta. On the other hand, the mean lymphocyte beta-adrenoceptor density was attenuated by 27%, showing significant difference between the acyanotic and the patent ductus arteriosus groups, but none between acyanotic and cyanotic or cyanotic and the patent ductus arteriosus groups. Among the congenital groups, only the left-to-right shunts and the pulmonary stenosis group showed significant (p < 0.05) decrease in beta-adrenoceptor density, whereas the affinity of all the groups toward [125I]iodocyanopindolol was hardly influenced. The plasma levels of all three catecholamines, norepinephrine, epinephrine, and dopamine, were elevated, but cAMP remained unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Reopening of a previously totally occluded ductus arteriosus with the Rashkind umbrella device occurred in a 5-yr-old girl, a previously unreported occurrence. Total occlusion using a second device was successful. This report emphasizes the need for regular follow-up with color flow Doppler echocardiography to diagnose and exclude a residual ductus even when apparent early closure has occurred.