Towards the definition of Institutional diagnostic reference levels in paediatric interventional cardiology procedures in Greece.

This study aimed to evaluate paediatric radiation doses in a dedicated cardiology hospital, with the objective of characterising patterns in dose variation. The ultimate purpose was to define Local (Institutional) Diagnostic Reference Levels (LDRLs) for different types of paediatric cardiac interventional procedures (IC), according to patient age. From a total of 710 cases performed during three consecutive years, by operators with more than 15 years of experience, the age was noted in only 477 IC procedures. The median values obtained for Fluoroscopy Time (FT), Number of Frames (N) and Kerma Area Product (PKA) by age range were 5.8 min, 1322 and 2.0 Gy.cm2 for <1 y; 6.5 min, 1403 and 3.0 Gy.cm2 for 1 to <5 y; 5.9 min, 950 and 7.0 Gy.cm2 for 5 to <10 y; 5.7 min, 940 and 14.0 Gy.cm2 for 10 to <16 y, respectively. A large range of patient dose data is observed, depending greatly on procedure type and patient age. In all age groups the range of median FT, N and PKA values was 3.1-15.8 min, 579-1779 and 1.0-20.8 Gy.cm2 respectively. Consequently, the definition of LDRLs presents challenges mainly due to the multiple clinical and technical factors affecting the outcome. On the other hand the lack of paediatric IC DRLs makes the identification of good practices more difficult. A consensus is needed on IC procedures nomenclature and grouping in order to allow a common assessment and comparison of doses.

Tumor-like pulmonary sarcoidosis diagnosed by CT-guided transthoracic lung biopsy.

INTRODUCTION: Most patients referred for lung biopsy have a focal lesion that is likely to be a carcinoma and fine needle aspiration (FNA) is usually sufficient to confirm diagnosis. Percutaneous cutting needle biopsy (CNB) is an important and potential diagnostic technique when non carcinomatous disease is suspected or when the pulmonary disease is unclear, so tissue architecture is very important. CASE PRESENTATION: We present a case of a 24 year old male arrived at our hospital with dyspnea and unusual computed tomography (CT) findings of sarcoidosis. Chest X-ray and CT scan revealed multiple masses in both lungs suggesting lung metastasis. Bronchoscopy and bronchoalveolar lavage did not reveal any malignant cells. None of the laboratory examinations revealed any primary extrapulmonary tumor. The patient underwent CT-guided core needle biopsy. Histopathological examination confirmed the diagnosis of sarcoidosis. CONCLUSION: CT-guided core needle biopsy is a very helpful diagnostic tool in order to determine the benign or malignant nature of a thoracic lesion.

Long-term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: a 2-year study.

OBJECTIVE: To evaluate the long-term clinical and exercise effect of chronic oral administration of the non-selective endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). DESIGN: Extension of a preceding prospective non-randomised open clinical study on bosentan treatment in PAH related to CHD. SETTING: A tertiary referral centre for cardiology. PATIENTS: 19 of the original 21 patients of mean (standard deviation (SD)) age 22 (3) years (13 with Eisenmenger syndrome) in World Health Organization (WHO) class II-IV and having a mean (SD) oxygen saturation of 87 (2) %. INTERVENTION: Patients received bosentan treatment for 2.4 (0.1) years and underwent clinical and exercise evaluation at baseline, 16 weeks and 2 years of treatment, with haemodynamic assessment at baseline and 16 weeks. RESULTS: All patients remained stable with sustained subjective clinical and WHO class improvement (p<0.01) at 16 weeks and 2 years of treatment without significant side effects or changes in oxygen saturation. After the initial 16-week improvement (p<0.05) in peak oxygen consumption and exercise duration at treadmill test, and walking distance and Borg dyspnoea index at 6-min walk test, all exercise parameters appeared to return to their baseline values at 2 years of follow-up. CONCLUSIONS: Long-term bosentan treatment in patients with PAH related to CHD is safe and induces clinical stability and improvement, but the objective exercise values appear to slowly return to baseline. Larger studies on long-term endothelin receptor antagonism including quality of life assessment are needed to evaluate the therapeutic role of bosentan in this population.

Effect of the oral endothelin antagonist bosentan on the clinical, exercise, and haemodynamic status of patients with pulmonary arterial hypertension related to congenital heart disease.

OBJECTIVE: To evaluate the clinical, exercise, and haemodynamic effects of chronic oral administration of the non-selective endothelin receptor antagonist bosentan on patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). DESIGN: Prospective non-randomised open clinical study. SETTING: Cardiology tertiary referral centre. PATIENTS: 21 patients with a mean (SEM) age of 22 (3) years with chronic PAH related to CHD (15 with Eisenmenger's syndrome). Patients were in World Health Organization (WHO) class II to IV with oxygen saturation 87 (2)%. INTERVENTION: Patients underwent clinical, exercise, and haemodynamic evaluations at baseline and after 16 weeks of treatment. RESULTS: Bosentan improved (p < 0.01) WHO class, peak oxygen consumption from 16.8 (1.4) to 18.3 (1.4) ml/kg/min, exercise duration from 9.0 (0.8) to 10.7 (0.6) minutes during the treadmill test, walking distance from 416 (23) to 459 (22) m, and Borg dyspnoea index from 2.8 (0.2) to 2.0 (0.1) during the six minute walk test. Bosentan treatment improved (p < 0.05) mean pulmonary artery pressure from 87 (4) to 81 (4) mm Hg, pulmonary blood flow index from 3.2 (0.4) to 3.7 (0.5) l/min/m2, pulmonary to systemic blood flow ratio from 1.2 (0.2) to 1.4 (0.2), and pulmonary vascular resistance index from 2232 (283) to 1768 (248) dyn.s.cm(-5). Two patients died, presumably of arrhythmic causes, who were in WHO class IV at baseline and who had improved during treatment. CONCLUSIONS: Bosentan induces short and mid term clinical, exercise, and haemodynamic improvements in patients with PAH related to CHD. Larger studies with long term endothelin receptor antagonism are needed to assess the safety and possible treatment role of bosentan in this population.

Acute endothelin A receptor antagonism improves pulmonary and systemic haemodynamics in patients with pulmonary arterial hypertension that is primary or autoimmune and related to congenital heart disease.

OBJECTIVE: To evaluate the acute haemodynamic effect of BQ-123, a selective endothelin A receptor antagonist, in severe chronic pulmonary arterial hypertension (PAH) of primary or autoimmune origin or related to congenital heart disease. DESIGN: Prospective open clinical study. SETTING: Cardiology tertiary referral centre. PATIENTS: 26 patients with chronic PAH were studied, with mean (SEM) age 29 (3) years (range 4-71 years), mean pulmonary artery pressure 68 (4) mm Hg, and pulmonary vascular resistance index 1694 (170) dyne x s x cm(-5). Patients were divided in three groups according to PAH aetiology: primary or autoimmune PAH (n = 12), and PAH associated with congenital heart defects with (n = 6) or without (n = 8) complete mixing. INTERVENTION: BQ-123 200 nmol/min was infused for 60 minutes in the right atrium with sequential haemodynamic measurements at 30 minute intervals. RESULTS: BQ-123 improved mean pulmonary artery pressure from 68 (4) to 64 (4) mm Hg (p < 0.05), pulmonary vascular resistance index from 1694 (170) to 1378 (145) dyne x s x cm(-5) (p < 0.001), pulmonary cardiac index from 3.0 (0.2) to 3.4 (0.3) l/min/m2 (p < 0.001), and effective cardiac index from 2.5 (0.2) to 2.7 (0.2) l/min/m2 (p < 0.01). Haemodynamic response was similar in all groups except for systemic cardiac index where a different (p = 0.0001, F = 5.53) response was observed; systemic cardiac index increased from 2.7 (0.2) to 2.9 (0.2) l/min/m2 (p < 0.001) when patients with complete mixing were excluded, in whom systemic cardiac index tended to decrease from 3.4 (1.0) to 3.0 (0.6) l/min/m2 (p = 0.06). CONCLUSIONS: Acute endothelin A receptor antagonism induces substantial haemodynamic improvement in severe chronic PAH of primary or autoimmune origin or related to congenital heart disease.

Diagnosis and management of pulmonary arteriovenous malformations.

Pulmonary arteriovenous malformation is a rare anomaly that presents in several different ways. It can present as an isolated finding, or more often in the context of hereditary haemorrhagic telangiectasia. It can also complicate palliative surgery such as the Glenn operation for complex congenital heart disease with single ventricle physiology. Its management includes transcatheter embolization, which is the preferred mode of therapy, surgery (including resection of the affected lobe, segment, or the fistula itself), or rarely, medical therapy. Complications of the disease itself and of various modes of treatment are relatively common, and patients require close surveillance for possible recurrence, or development of new fistulas. In cases related to the Glenn operation, redirection of hepatic venous flow or heart transplantation may cure the problem.

Transcatheter occlusion of a large pulmonary arteriovenous malformation with use of a Cardioseal device.

Large pulmonary arteriovenous malformations (PAVMs) carry a significant risk of neurologic complications and present technical difficulties in transcatheter treatment with use of coils or detachable balloons. A 26-year-old man with a giant PAVM, who had undergone unsuccessful attempted closure with use of a Gianturco-Grifka occlusion device in the past, underwent successful transcatheter embolization with two Cardioseal double umbrella devices designed for occlusion of intracardiac communications. The procedure was technically easy, had no complications, and provided sustained improvement in arterial saturation and exercise tolerance during follow-up. Transcatheter double umbrella device occlusion of large arteriovenous malformations is feasible and should be considered, especially for very large fistulas.

Transcatheter occlusion of atrial baffle leak after mustard repair.

We present a case of transcatheter closure of an atrial baffle leak with significant systemic to pulmonary atrium shunt in a patient late after Mustard operation and pulmonary valvotomy for transposition of the great arteries. This procedure alleviated the need for reoperation in a high-risk symptomatic patient. Cathet. Cardiovasc. Intervent. 51:305-307, 2000.

One-lung Fontan operation: hemodynamics and surgical outcome.

BACKGROUND: This study examined the results of a Fontan operation for patients with acquired atresia of one main branch pulmonary artery. METHODS: The data for 7 patients identified as having a hypoplastic left pulmonary artery discontinuous from the right pulmonary artery were compared with those for 65 patients with continuous pulmonary arteries who consecutively underwent a completion Fontan procedure. RESULTS: No significant differences were found preoperatively with respect to right atrial pressure, aortic saturation, ventricular end-diastolic pressure, pulmonary artery pressure, pulmonary blood flow, or pulmonary vascular resistance. In the first 24 postoperative hours, there were no significant differences in heart rate, urine output, systemic venous pressure, or pulmonary venous pressure. Also, data regarding hospitalization length, effusions, and mortality were similar between the two groups. Postoperative systemic arterial saturation was lower in the one-lung group. There were no early postoperative deaths in the one-lung group, and 5 of the 7 patients are long-term survivors. CONCLUSIONS: A completion Fontan procedure can be successfully performed in patients with a hypoplastic and discontinuous left pulmonary artery, although postoperative systemic arterial saturation is not as high as in patients with continuous pulmonary arteries.

Early reduction of the volume work of the single ventricle: the hemi-Fontan operation.

BACKGROUND: In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume work associated with parallel pulmonary and systemic circulations. The hemi-Fontan operation was conceived as a means of accomplishing early reduction of the volume work of the single ventricle. METHODS: All patients presenting in infancy with single-ventricle physiology were managed by early hemi-Fontan operation in anticipation of a subsequent completion Fontan operation. Between May 1989 and August 1995, 400 patients less than 2 years of age underwent hemi-Fontan operations. Mean age at operation was 8.5 months (range, 2 months to 24 months). The hemi-Fontan operation included association of superior vena(e) cava(e) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the superior vena cava into the right atrium, and elimination of other sources of pulmonary blood flow. RESULTS: Operative mortality ( < 30 days) was 31 of 400 patients (7.8%). For the last 200 patients, operative mortality was 8 of 200 (4.0%). Younger age at operation was not an independent risk factor for operative mortality. Urgent operation in the presence of a hemodynamic burden requiring concomitant procedures was associated with increased mortality. CONCLUSIONS: The hemi-Fontan operation can be accomplished with low operative mortality in young patients, achieving early reduction of the volume work of the single-ventricle heart.