Transcatheter occlusion of a left ventricular to right atrial communication by an Occlutech duct occluder.

Left ventricular to right atrial communication is a rare congenital or acquired heart defect. There are many reports of successful transcatheter closure of this defect. We describe the device closure of one such communication in a 5-year-old girl using the Occlutech duct occluder. We believe that this device may have some advantages over the devices previously used for this purpose.

Neonatal marfan syndrome: report of two cases.

BACKGROUND: Neonatal Marfan syndrome is a rare and severe phenotype of this disease. A poor prognosis is anticipated due to the high probability of congestive heart failure, and mitral and tricuspid regurgitations with suboptimal response to medical therapy and difficulties in surgical management at an early age. CASE PRESENTATION: We present two consecutive patients with this disease who are the first reported cases from Iran to the best of our knowledge. Unfortunately both of them died shortly after diagnosis. CONCLUSION: Neonatal Marfan syndrome is reported from Iran and has a poor prognosis like the patients reported from elsewhere.

Intracoronary administration of autologous mesenchymal stem cells in a critically ill patient with dilated cardiomyopathy.

Relatively high prevalence of dilated cardiomyopathy in children, unfavorable response to traditional drug therapy, and limitations in heart transplantation call for new therapeutic options. Stem cell therapy can be promising in children suffering from this disease. The presented case documents that intracoronary injection of autologous bone marrow-derived mesenchymal stem cells in a boy with progressive dilated cardiomyopathy is feasible and safe. Furthermore, it may positively influence functional class, quality of life, and echocardiographic indices of cardiac function.

Ross operation in a neuro-cardio-facial-cutaneous syndrome patient.

Congenital heart diseases are a major part of Costello and cardio-facio-cutaneous syndromes. Subaortic stenosis was reported rarely and Ross operation never in these syndromes.We reported a girl patient whose manifestations were consistent with these syndromes. Distinction between these syndromes was not possible as genetic testing was not carried out. She developed severe neoaortic regurgitation 2.5 years after the Ross operation and died due to the complications of aortic valve replacement.Ross operation may be an unsuitable option in these syndromes due to the possibility of subtle pulmonic valve pathology.

Device closure of patent ductus arteriosus in Marfan patients: safety and effect on the aortic root diameter.

OBJECTIVES: There is little evidence in the medical literature about safety of PDA device closure and its effects on aortic root diameter in Marfan patients. We reported on nine Marfan patients whose ducts were occluded by coil or Amplatzer Duct Occluder. PATIENTS AND INTERVENTIONS: Two patients had aneurismal type E ducts which were closed by a single coil, respectively. The others had conical ducts for which even Amplatzer occluder, stiff, or double coils were used. RESULTS: No untoward consequence was found during a median follow up period of 18.5 months (range 3 months to 6.33 years). Two patients had small residual shunts. The observed change in the mean ratio of aortic root diameter to its estimated value was statistically insignificant in seven patients who had complete data on their aortic root diameter before PDA closure and during the follow up. CONCLUSIONS: We concluded that PDA device closure in Marfan patients is safe. Risk of residual shunt is higher even after closure of small ducts.

Feasibility and efficacy of stent redilatation in aortic coarctation.

OBJECTIVES: We evaluated the efficacy and safety of stent redilation in patients with aortic coarctation (COA). METHODS AND RESULTS: In our retrospective study of 28 stent redilation procedures on 22 patients, the interval between implantation and redilation ranged from 2.5 month to 11 years (median 18 months). Ten patients had native COAs, one had repaired interrupted aortic arch. The remaining patients had residual COAs after surgery. Stent redilation was successful in 26 procedures. Pressure gradients decreased from a mean of 26 +/- 13.8 mm Hg to 7.9 +/- 6.52 mm Hg (P < 0.001). The mean diameter of the stents increased from 9.19 +/- 3.29 mm to 12.1 +/- 3.68 mm (P < 0.001). In a patient with Williams-Beuren syndrome, massive neointimal proliferation made stent redilation impossible. One other patient developed aortic aneurysm after redilation and was operated after 3 years. CONCLUSIONS: Stent redilation for aortic COA is a safe and successful procedure.