Maria Zubrzycka

Background: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfa­vourable prognosis. Aim: To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004–2013. Methods: The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH — n = 23 (42%), PAH associated with systemic-to-pulmonary shunts — n = 17 (31%), and PAH after corrective cardiac surgery — n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy. Results: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03). Conclusions: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

A Fontan-type operation, i.e. a connection of the systemic veins and pulmonary arteries without subpulmonary ventricle, with different surgical techniques, is nowadays the only treatment option for patients with a functionally univentricular heart (UVH). Understanding the development of pulmonary arteries in patients who are considered for the Fontan procedure is important clinically. To evaluate the development of pulmonary arteries in patients with univentricular circulation. Between 1995 and 2007, 111 patients underwent a bidirectional Glenn procedure. In all patients, preoperative catheterisation was performed to assess the anatomy and haemodynamics of UVH, especially the size of the pulmonary arteries. Ninety nine patients were included in the bidirectional Glenn group; 62 of these underwent repeat catheterisation before Fontan completion. The late results, after one stage extracardiac total cavopulmonary anastomosis performed in 24 patients between 1992 and 2002, were reinvestigated (one-stage Fontan group). We assessed the changes in the McGoon ratio and Nakata index for the whole cohort of patients. McGoon ratio is the sum of the diameter of pulmonary arteries divided by the diameter of the aorta. Nakata index is the sum of the cross-sectional area of the pulmonary arteries divided by the body surface area. During cardiac catheterisation prior to Glenn procedure, the mean Nakata index was 351.9 (range 131.2-886) mm2/m2 and was higher in patients with increased pulmonary flow (p = 0.0135). Mean McGoon ratio was 2.5 (range 1.1-4.9). An average 40.3 months after Glenn procedure, the Nakata index and McGoon ratio decreased significantly to 226.4 ± 125 mm²/m² (p < 0.003), and to 2.14 ± 0.58 (p < 0.008) respectively. In the group of patients after one-stage Fontan in late follow-up, mean 7.4 years after procedure, the Nakata index decreased from 318.7 ± 159.1 mm²/m² to 120 ± 40 mm²/m² (p < 0.0001) and McGoon ratio from 2.4 ± 0.6 to 1.4 ± 0.27 (p < 0.0001). Only size of pulmonary arteries before Glenn procedure, in the bidirectional Glenn group, or before Fontan operation, in the one-stage Fontan group,were inversely correlated with the changes of size of pulmonary arteries (p = 0.0015 and p = 0.0012). The relative decrease of the size of pulmonary arteries in the inter-stage period (between bidirectional Glenn anastomosis and Fontan completion) and after Fontan completion may indicate that pulmonary artery sizes should probably not bean absolute limiting factor in the decision on treatment of functionally UVH patients, especially at the stage of Fontan approach.

Modified Blalock–Taussig (B–T) shunt occlusion results in a sudden reduction of pulmonary vascular perfusion, causing dramatic saturation drop and cy

We inserted covered Cheatham-Platinum stents in 4 patients, ranging in age from 12 to 19 years, who weighed between 45 and 94 kg. All the patients had aortic coarctation, with surgical repair having been attempted previously in one, and with balloon dilation having been performed as the primary treatment in two, resulting in formation of aneurysms. The fourth patient had not received any treatment. The gradients were reduced from 10 to 40 mmHg before insertion of the stent to 0 to 5 mmHg after stenting. No complications were encountered. All the patients are well at an interval of 3 to 14 months after stenting.

OBJECTIVE To determine the early results of balloon expandable stent implantation for aortic coarctation or recoarctation. DESIGN Prospective observational study. SETTING Two paediatric cardiology tertiary referral centres. PATIENTS 17 patients, median age 17 years (range 4.4 to 45) and median weight 61 kg (17 to 92). Six had native aortic coarctation and 11 had aortic recoarctation; 14 had upper limb systolic hypertension. Of those with recoarctation, eight had had at least one previous balloon dilatation attempt and two of these patients also had further surgical interventions. INTERVENTION Balloon expandable Palmaz iliac stent implantation. MAIN OUTCOME MEASURES Systolic pressures gradients, minimum aortic diameter, upper limb blood pressures, and incidence of aneurysm formation. RESULTS 18 stents were implanted during 18 procedures in the 17 patients. Mean peak systolic pressure gradient fell from 26 mm Hg (95% confidence interval (CI), 21 to 31 mm Hg) before to 5 mm Hg (2 to 8 mm Hg) after stent implantation (p < 0.001), and mean minimum aortic diameter increased from 7 mm (95% CI, 6 to 8 mm) before to 11.3 mm (10 to 12.6 mm) after implantation (p < 0.001). Complications occurred in five patients (bleeding in two, stent migration in two, and aneurysm formation in one). Two patients remained borderline hypertensive and eight were receiving antihypertensive treatment at most recent assessment. CONCLUSIONS Stent implantation for aortic recoarctation and native coarctation gives good immediate results. Careful follow up is necessary to evaluate complications and the long term effect on blood pressure.

Objectives To determine outcome of stent implantation in patients with middle aortic syndrome. Design Prospective study, case series. Setting A tertiary paediatric cardiology centre in a children’s hospital. Patients Five patients, aged 4 to 17 years (mean 11.4 years), with upper limb hypertension due to middle aortic syndrome. Intervention Stents were implanted in the mid/lower thoracic/upper abdominal aorta. Main outcome measure Satisfactory deployment of stents and blood pressure control. Results In all patients, angiocardiography showed long segment stenosis in the mid or lower thoracic/upper abdominal aorta. The pressure gradient was between 40 and 90 mm Hg (mean 63.2 mm Hg). Seven Palmaz stents were implanted. Immediately after implantation, the gradient decreased to between 0 and 35 mm Hg (mean 13.6 mm Hg). Angiography showed a satisfactory result with widely patent stents in all. In one patient, thrombosis of the stent occurred six days after implantation. This was successfully treated with infusion of alteplase, further balloon dilatation, and implantation of a second stent overlapping the first, both dilated to 10 mm diameter. One patient had elective redilatation of the stent six months after implantation, with further reduction of the gradient from 35 mm Hg to 10 mm Hg. At the latest follow up between three and 20 months (mean 12.2 months) after stent implantation, in four patients blood pressure was better controlled with antihypertensive drugs. One patient was normotensive without drugs. Computed tomography showed no aneurysm formation in the region of the stents. Conclusions Stent implantation is a preferable alternative to surgery in the treatment of patients with middle aortic syndrome and merits further evaluation.