Role of echocardiography in the assessment of adolescents and adults with congenital heart disease.

Adolescent and adults with congenital heart disease (CHD) represent a special group of individuals. They often require regular assessment of their cardiac anatomy and functional hemodynamics. Most children with repaired CHD surviving to adulthood are either corrected or fully repaired and may not require long term follow-up. However, there are is a significant number of grown up children with residual abnormality or palliated defects, in whom full correction was not possible. Some of them are likely to present with complications and need repeated evaluation. Others may require improvement or stabilisation of their hemodynamics during stressful period like pregnancy or non-cardiac surgery. These conditions include single ventricle repair, repaired tetralogy, Mustard or Sennings operation for transposition of great arteries, aortic arch abnormalities, postatrioventricular septal defect (AVSD) repair, bicuspid aortic valve, Ebstein anomaly and unoperated congenital heart disease leading to Eisenmenger syndrome. The focus of this discussion will be mainly on this subset.

3-D echocardiographic imaging of double aortic arch.

Delineation of arch abnormalities is difficult by conventional 2-D echocardiography and MRI has been the investigation of choice. 3-D echocardiography is increasingly used in congenital heart disease for both functional anatomy and morphology. This case report demonstrates that 3-D echocardiography can be used in delineating arch anomalies, which can avoid further imaging that needs a general anaesthetic.

Acquired combined immunodeficiency associated with protein losing enteropathy complicating Fontan operation.

It is vitally important that the immunological aspect of protein losing enteropathy following Fontan procedures is highlighted, in order to decrease significant morbidity and mortality

Pulmonary vein stenosis mimicking chronic lung disease.

UNLABELLED: The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography. CONCLUSION: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.

Safe retrieval of impacted central venous line.

Embolisation of impacted central venous lines is not uncommon in paediatric practice. We describe a method of preventing this complication and associated risks.

Congenital coronary artery dilatation.

Although dilated coronary arteries are uncommon in children, three cases are presented which, even more unusually, were not associated with Kawasaki's disease

Balloon angioplasty in infants with aortic obstruction after the modified stage I Norwood procedure.

BACKGROUND: The incidence of coarctation after stage I Norwood procedure varies between 11% and 37%, and it contributes to late death after this operation. We describe the incidence and report the results of percutaneous balloon angioplasty of neoaortic arch obstruction in patients after the modified Norwood procedure for hypoplastic left heart syndrome (HLHS). METHODS: During a period of 5 years, 136 patients (94 male, 42 female) underwent a first stage modified Norwood procedure for HLHS. All 95 survivors (69.8%) underwent cardiac catheterization before the second stage. Neoaortic arch obstruction was diagnosed on documentation of a peak systolic gradient of >10 mm Hg across the arch associated with angiographic evidence of localized narrowing of the aortic lumen. RESULTS: Twenty-one (22.1%) of the 95 survivors were documented to have neoaortic arch obstruction. Seventeen patients underwent percutaneous balloon angioplasty for the relief of the neoaortic arch obstruction. The predilatation peak gradient across the arch was reduced significantly by angioplasty from 28.6 +/- 16.9 mm Hg (range 10 to 73 mm Hg) to 5.3 +/- 5.2 mm Hg (range 0 to 19 mm Hg) (P <.001). A final gradient <10 mm Hg or <70% of the starting gradient was considered a success. CONCLUSION: The absence of serious sequelae after the procedure or need for reintervention, as shown by our study, makes balloon angioplasty an effective technique and the treatment of choice for the relief of recoarctation of the neoaortic arch in patients with staged palliation of HLHS.

Intermittent complete closure of the arterial duct.

Intermittent functional closure of a patent duct has been reported both clinically and echocardiographically. We describe the case of a 6-year-old with intermittent complete closure of a patent duct occurring during attempted transcatheter closure. Recurrence of clinical and echocardiographic signs prompted restudy. A patent duct of moderate size was demonstrated and successfully occluded by coil embolization. The interventional aspects of this unusual condition are discussed.

Intrapulmonary arteriovenous shunting may be a universal phenomenon in patients with the superior cavopulmonary anastomosis: a radionuclide study.

OBJECTIVE: To evaluate the extent of intrapulmonary right to left shunting in children after bidirectional cavopulmonary anastomosis (BCPA). DESIGN: Prospective study of patients who underwent BCPA in a single centre. PATIENTS: 17 patients with complex cyanotic congenital cardiac malformations who underwent BCPA at 1-45 months of age (median 21 months) were evaluated 15-64 months postoperatively (median 32 months). Five children between 1 and 10 years (median 5 years) with normal or surgically corrected intracardiac anatomy and peripheral pulmonary circulation who required V/Q scanning for other reasons were used as controls. INTERVENTIONS: All patients underwent cardiac catheterisation to exclude angiographically demonstrable venovenous collaterals followed by pulmonary perfusion scanning using (99m)technetium ((99m)Tc) labelled albumen microspheres to quantify the intrapulmonary right to left shunt. MAIN OUTCOME MEASURE: Percentage of intrapulmonary right to left shunt. RESULTS: The mean (SD) level of physiological right to left shunting found in the control group was 5.4 (2.3)%. All patients with BCPA showed the presence of a significantly higher level of intrapulmonary shunting (26.8 (16.9)%, p < 0.001). The degree of shunting was significantly increased in the subgroup of 11 patients with BCPA as the only source of pulmonary blood flow (34.9 (15.8)%), when compared to the six remaining patients with an additional source of pulmonary blood supply (12.0 (2.6)%, p < 0.001). There was a negative correlation between age at BCPA and the shunt percentage found in the patients with a competitive source of pulmonary blood flow (r = -0.63, p < 0. 01). CONCLUSIONS: Intrapulmonary right to left shunting develops in all patients following BCPA. This may be caused by a sustained and inappropriate vasodilatation resulting from absence or decreased levels of a substance that inhibits pulmonary vasodilatation. Augmenting BCPA with an additional source of blood flow containing hepatic factor limits the degree of intrapulmonary arteriovenous shunting and may help provide successful longer term palliation.