John W. Moore

Percutaneous closure of the small (< 2.5 mm) patent ductus arteriosus using coil embolization

Persistently patent ductus arteriosus (PDA) is a common form of congenital heart disease, occurring between 0.01 to 0.08% of live births.1 Surgical ligation by lateral thoracotomy is effective, yet carries procedural risks (potential bleeding, recurrent laryngeal nerve injury, exposure to general anesthesia and death [<1%]).2 In 1971, Porstmann et al3 described the first nonsurgical PDA closure with an Ivalon plug with an 18Fr arterial conduit. More recently, the Rashkind PDA double disc occlusion system has undergone extensive evaluation, with flow occlusion accomplished in 72 to 88% of cases attempted.4–6 The smaller Rashkind occluder requires an 8Fr delivery system. Rao et al7 described an occluder which may be introduced through a 7Fr catheter and reduces the crossing profile required. Nevertheless, technical limitations persist in patients whose PDA internal diameter is <2.5 mm. n nTechniques to facilitate cannulation of the very small ductus by the Rashkind delivery system, involving arterial cannulation and rendezvous of an exchange wire from femoral vein to contralateral artery,8 and ductal dilation with balloon angioplasty,9 have been described. These techniques require additional manipulations and procedural time, and may potentially increase patient morbidity. This report describes experience with an alternative technique for transcatheter closure of the very small ductus arteriosus using stainless steel coil embolization.

Use of an intravascular endoprosthesis (stent) to establish and maintain short-term patency of the ductus arteriosus in newborn lambs.

The feasibility of stenting the ductus arteriosus with a balloon-expandable vascular endoprosthesis was tested in 8 newborn lambs. Tantalum wire and stainless steel mesh coronary stents were implanted antegrade or retrograde by percutaneous transfemoral catheterization. One lamb died during the procedure from perforation of the aorta. In 7 lambs, the ductus arteriosus was crossed using endhole catheters and wires, and stents mounted on angioplasty catheters were expanded in the ductus arterisus. Six lambs had successful implantation and had maintained a sizeable patent ductus arteriosus at 2 h. We conclude that the feasibility of percutaneous stenting of the newborn ductus was demonstrated. By providing patency of the ductus arteriosus, stents may offer nonsurgical alternatives for palliation of cyanotic congenital heart disease and hypoplastic left heart syndrome

The inheritance of conotruncal malformations: a review and report of two siblings with tetralogy of Fallot with pulmonary atresia.

Congenital heart defects (CHD) are a group of structural abnormalities that in humans have a combined incidence of approximately 1%. It is estimated that 4–5% of CHD are associated with chromosome abnormalities, 1–2% are associated with single gene syndromes, 1–2% are due to known teratogens, with the rest presumably determined multifactorially. We report on a brother and sister with tetralogy of Fallot with pulmonary atresia, and review the inheritance of familial conotruncal anomalies. We feel the small number of family clusters and the rare instances of consanguinity in non‐syndromal conotruncal defects are consistent with multifactorial determination. While it is prudent in counseling families with 2 or more individuals with conotruncal CHD to raise the possibility of single gene inheritance, we believe that current empiric recurrence risk estimates most accurately reflect their risks.

Results of repeat percutaneous balloon valvuloplasty for pulmonary valvar restenosis

Follow-up cardiac catheterization studies were used to evaluate 105 patients who had undergone percutaneous balloon pulmonary valvuloplasty. Fifteen of those patients who had peak systolic pulmonary valve gradients greater than = 40 mm Hg at follow-up underwent repeat balloon valvuloplasty. For the initial balloon pulmonary valvuloplasty, the mean ratio of the balloon diameter to pulmonary valve annulus diameter was 0.98 +/- 0.2; at repeat valvuloplasty the mean was 1.19 +/- 0.12. The immediate post-repeat balloon valvuloplasty results showed a reduction in the peak systolic gradient from a mean of 70.2 +/- 17.8 to 29.1 +/- 19.0 mm Hg (p less than 0.001). This reduction in the gradient was maintained at a mean of 14.3 +/- 5.0 mm Hg in 8 of the 10 patients who underwent further follow-up studies. We conclude that successful repeat balloon pulmonary valvuloplasty with the use of larger sized balloons is feasible in patients who have restenosis after the initial percutaneous balloon valvuloplasty--including partial but not complete dysplasia of the pulmonary valve.

Gastrointestinal hemorrhage after combined percutaneous angioplasty of aortic coarctation and valvuloplasty of aortic stenosis in an infant.

SummaryGastrointestinal hemorrhage has not been previously reported as a complication of dilating left-sided obstructive lesions. This report describes an infant who developed significant intestinal bleeding after combined angioplasty for aortic coarctation and valvuloplasty for aortic stenosis.

Necrotizing arteritis in uncorrected tetralogy of fallot with pulmonary atresia

SummaryA 10-year-old girl with uncorrected tetralogy of Fallot with pulmonary atresia presented with fevers of unknown origin and left lung infiltrates. At autopsy, necrotizing vascular changes resembling those of severe pulmonary hypertension (grade VI in the Heath-Edwards classification) were confined to the left lung. Pulmonary blood flow and pressure were greater in the left lung and were provided by an enlarged collateral artery arising directly from the descending thoracic aorta. To our knowledge, this is the first report of necrotizing arteritis of the pulmonary arteries in uncorrected tetralogy of Fallot with pulmonary atresia.

Tolerance of normal aorta to oversized dual balloon valvuloplasty. Observations in a swine model: Technical note

The damage threshold during aortic valvuloplasty was determined in 12 normal swine subjected to inflation of oversized dual balloons. Catheters with combined balloon diameters of 1.2–2.0 times the aortic annulus were selected. Following completion of the procedure, the animals were sacrificed and examined for pathology. With combined balloon diameters less than or equal to 1.7 times the aortic annulus, there was no gross, or microscopic damage. In animals with combined diameters equal to 2 times the aortic annulus, there was rupture of the aorta. This data provides further basis for the selection of catheter sizes in dual balloon aortic valvuloplasty.

Aortic root dilatation associated with partial trisomy 7(q31.2→qter)

SummaryAortic root dilatation and mitral valve prolapse are cardiac findings sometimes seen in disorders of connective tissue, most often in the Marfan syndrome. This report describes an infant with these cardiac anomalies and a specific chromosomal abnormality, partial trisomy of chromosome 7 associated with partial monosomy of chromosome 22. This association may have significance with respect to the etiology of cardiac disease in connective tissue disorders such as Marfan syndrome.