Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

BACKGROUND: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. METHODS: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. RESULTS: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). CONCLUSIONS: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

Aneurysms Associated with Brain Arteriovenous Malformations.

Brain arteriovenous malformations are frequently associated with the presence of intracranial aneurysms at a higher-than-expected incidence based on the frequency of each lesion individually. The identification of intracranial aneurysms in association with AVMs has increased due to improvement in diagnostic techniques, particularly 3D and superselective conventional angiography. Intracranial aneurysms may confer a higher risk of hemorrhage at presentation and of rehemorrhage in patients with AVMs and therefore may be associated with a more unfavorable natural history. The association of AVMs and intracranial aneurysms poses important therapeutic challenges for practicing neurosurgeons, neurologists, and neurointerventional radiologists. In this report, we review the classification and radiology of AVM-associated intracranial aneurysms and discuss their clinical significance and implications for treatment.

Changing Clinical and Therapeutic Trends in Tentorial Dural Arteriovenous Fistulas: A Systematic Review.

BACKGROUND AND PURPOSE: Tentorial dural arteriovenous fistulas are characterized by a high hemorrhagic risk. We evaluated trends in outcomes and management of tentorial dural arteriovenous fistulas and performed a meta-analysis evaluating clinical and angiographic outcomes by treatment technique. MATERIALS AND METHODS: We performed a comprehensive literature search for studies on surgical and endovascular treatment of tentorial dural arteriovenous fistulas. We compared the proportion of patients undergoing endovascular, surgical, and combined endovascular/surgical management; the proportion of patients presenting with ruptured tentorial dural arteriovenous fistulas; and proportion of patients with good neurologic outcome across 3 time periods: 1980-1995, 1996-2005, and 2006-2014. We performed a random-effects meta-analysis, evaluating the rates of occlusion, long-term good neurologic outcome, perioperative morbidity, and resolution of symptoms for the 3 treatment modalities. RESULTS: Twenty-nine studies with 274 patients were included. The proportion of patients treated with surgical treatment alone decreased from 38.7% to 20.4% between 1980-1995 and 2006-2014. The proportion of patients treated with endovascular therapy alone increased from 16.1% to 48.0%. The proportion of patients presenting with ruptured tentorial dural arteriovenous fistulas decreased from 64.4% to 43.6%. The rate of good neurologic outcome increased from 80.7% to 92.9%. Complete occlusion rates were highest for patients receiving multimodality treatment (84.0%; 95% CI, 72.0%-91.0%) and lowest for endovascular treatment (71.0%; 95% CI, 56.0%-83.0%; P < .01). Long-term good neurologic outcome was highest in the endovascular group (89.0%; 95% CI, 80.0%-95.0%) and lowest for the surgical group (73.0%; 95% CI, 51.0%-87.0%; P = .03). CONCLUSIONS: Patients with tentorial dural arteriovenous fistulas are increasingly presenting with unruptured lesions, being treated endovascularly, and experiencing higher rates of good neurologic outcomes. Endovascular treatment was associated with superior neurologic outcomes but lower occlusion rates.

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.

Early results after transatrial/transpulmonary repair of tetralogy of Fallot.

OBJECTIVES: Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of tetralogy of Fallot (TOF). Transatrial/transpulmonary repair avoids a ventriculotomy (in contrast to the transventricular approach) emphasizing maximal preservation of RV structure and function. We have adopted this technique as less traumatic for the right ventricle. This study evaluates the early surgical results of our approach. METHODS: Between September 1997 and July 2001, 110 consecutive patients with TOF were referred to our unit for surgical therapy. Of these, 14 were unsuitable for repair and underwent aortopulmonary shunting+/-pulmonary artery patching. In the remaining 96 patients (median age 1.4 years), complete transatrial/transpulmonary repair was performed. Previously placed shunts (ten patients) were taken down and any secondary stenoses or branch pulmonary artery distortion repaired. In all cases, subpulmonary resection and ventricular septal defect (VSD) closure were accomplished transatrially. Whenever pulmonary valvotomy and valve ring widening were necessary, it was achieved through a pulmonary arteriotomy. In 84 patients the main pulmonary artery was augmented with an autologous pericardial patch, and in 23 the patch was extended to pulmonary artery branch(es). A limited (<1cm ) or extended (>1cm, but

Repair of critical aortic coarctation in neonatal age.

BACKGROUND: The data of 111 (male: 64; female: 47) in the period of 1967 until 12/93 consecutive operated neonatals (<1 month) were studied retrospectively (mean weight 3270 g, mean age at operation 14 days). METHODS: Preductal anatomy was present in 96 patients. The coarctation was isolated in 30 patients (group I), 34 patients had additional large ventricular septal defects (group II) and 47 had complex heart disease (group III). The preoperative heart catheterization revealed a gradient of <20 mmHg in 35%, >20 mmHg in 51.4% and >50 mmHg in 12.9%. The indication for repair was conservatively untreatable heart insufficiency. In the vast majority (n=97) of patients resection and end-to-end anastomosis were performed, in 31 cases using an absorbable suture, in 18 of these using a continuous suture line. In 4 patients a subclavian flap angioplasty (SFA) was done, in 4 a patch enlargement, 4 times a repair was described as not possible and in 2 patients there was no gradient after division of the ductus. RESULTS: Early lethality was 3.3% (n=1) in group I, 24.2% (n=8) died in group II and 39.1% (n=18) in group III; after introducing Prostaglandin E1 0% in group I, 15% in II and 25% in III. Relevant recoarctation (Gradient >20 mmHg) developed in 9 (among them 4 with hypoplastic arch, 2 after SFA) of the 77 long-term survivors; 6 of these were reoperated on, 5 without residual gradient, 1 with a gradient of 25 mmHg without clinical symptoms (after 4 years). In the last 3 patients a balloon dilation was carried out without residual gradient. Mean follow-up time was 6 (0-24) years. No patient needs antihypertensive treatment. The cumulative survival rate is 96.7% (+6.6%) for group I, 77.4% (+15.0%) for II and 51.9% (+16.6%) for III. CONCLUSIONS: Resection and end-to-end anastomosis using a continuous absorbable suture is the method of choice at theoretical considerations and in our experiences. The number of recoarctations in neonatal age is relatively high; reinterventions (operation respectively dilation) can be done safely and successfully.

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.

Long-term results after repair of total anomalous pulmonary venous connection.

BACKGROUND: Operative strategies and early results concerning repair of Total Anomalous Pulmonary Venous Connection (TAPVC) are relatively well known. Less well defined data are available to evaluate the long-term outcome. We would therefore like to contribute our long-term data in this presentation. PATIENTS AND METHODS: Between 1958 and 1992 52 consecutive patients aged two days to 42 years (15 neonates, 16 infants, 9 children and 12 adults) with TAPVC were operated on. The data were collected retrospectively from the records. In 24 patients, a current follow-up study was performed, including clinical evaluation, echocardiography, and a twenty-four-hour ambulatory ECG. RESULTS: Early mortality was 34.6% (n = 18). The postoperative follow-up period ranged from 4 months to 28 years (mean 10.7 years). There were 4 late deaths, yielding an overall long-term mortality of 7.7% (4/52). Causes of death were severe hypoplasia of central pulmonary veins in 1, ventricular fibrillation (2) and non-cardiac in one case. 80% of the operative survivors were available for assessment. Preoperatively, 11 of these patients were in NYHA functional class II, six in class III and seven in class IV. After treatment, 22 patients were in class I and two in class II. Ventricular function was evaluated by echocardiography and invasive catheterization. Only two of 24 patients (8%) showed an abnormal IVS-motion and enlargement of the right ventricle. Cardiac catheterization revealed a mean PA pressure of 26 mmHg, the peak systolic pressure in the RV was 34 mmHg. All 24 long-term survivors underwent assessment of cardiac rhythm by 24 h electrocardiogramm (ECG) monitoring. Significant arrhythmias were recorded in 11 of 24 cases (46%), including sinus node dysfunction in 3 patients. Multiform ventricular ectopic beats were evaluated in 9 cases. According to the Lown classification, 7 patients were class I while 2 cases were considered to be class IV. CONCLUSIONS: A normal hemodynamic state can be achieved in most cases. Significant arrhythmias may exist in asymptomatic patients late after surgical correction of TAPVC, and therefore, long-term follow-up of these patients, including 24 h ECG monitoring, is recommended, even if they are asymptomatic.

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.

Double-inlet and double-outlet left ventricle in situs inversus.

Double-outlet left ventricle is a rare congenital cardiac malformation that has been traditionally difficult to diagnose accurately. We report a unique case of situs inversus totalis, L-loop, double-inlet left ventricle and double-outlet left ventricle with pulmonary stenosis, diagnosed mainly by transesophageal echocardiography and magnetic resonance imaging.

Complete AV-block in two children with immunohistochemical proven myocarditis.

UNLABELLED: We report two children with acquired third degree AV-block caused by acute myocarditis. The diagnosis was proven by endomyocardial biopsy. Severe lymphocytic myocardial infiltration was shown using immunohistological methods. One of the children was treated with prednisone During therapy conduction disturbance nearly disappeared and infiltration was markedly reduced in a subsequent biopsy. In the other patient the parents refused immunosuppressive treatment and a permanent pacemaker was necessary for persistent bradycardia. CONCLUSION: Immunohistological staining of an endomyocardial biopsy can be used to establish the diagnosis of myocarditis in patients with atypical clinical manifestation, such as complete AV-block, and can support the decision for therapy. In one patient improvement was documented by the disappearance of inflammatory activity in a repeated biopsy.

Longterm efficiency of high dose inotropic support in an infant after repair of Fallot's tetralogy.

A 23 month old boy with highly symptomatic tetralogy of Fallot (TOF) underwent repair. Inspite of cold Bretschneider cardioplegic solution twice the heart was beating soon after application of the cardioplegic solution each time. Soon after transfer to the intensive care unit the patient developed low cardiac output (LCO). The following days high doses of inotropic support ware necessary to maintain sufficient arterial pressure. The dosages of dobutamine (up to 49 micrograms/kg/min); norepinephrine (up to 5.28 micrograms/kg/min, and epinephrine (up to 16 micrograms/kg/min), respectively, were twice and three times as high as common maximum recommendations. After having recovered from acute renal failure requiring hemodialysis from the 5th to the 37th postoperative day the child was discharged 9 weeks after the intervention. The very unusual and interesting course of this boy is described and the form and grade of the inotropic support is discussed.

Heparin as a cause of thrombus progression. Heparin-associated thrombocytopenia is an important differential diagnosis in paediatric patients even with normal platelet counts.

UNLABELLED: A 15-year-old boy developed deep vein thrombosis of the right leg 9 days after appendectomy. In spite of three courses of thrombolysis with streptokinase and effective heparinization the thrombosis progressed with additional occlusion of the left iliac vein. Although platelet counts were constantly normal, heparin-associated thrombocytopenia was suspected as the cause of the new venous occlusions. This diagnosis was confirmed by detecting heparin-associated antibodies with the heparin-induced platelet activation test. Therapy was instituted replacing heparin by the low molecular weight heparinoid Orgaran. Bilateral recanalization occurred within 6 days. CONCLUSION: Heparin-associated thrombocytopenia must be considered if thrombosis occurs or progresses despite effective heparinization even in the absence of thrombocytopenia.

Evaluation of low dose prostaglandin E1 treatment for ductus dependent congenital heart disease.

UNLABELLED: This study reports our experience with low-dose prostaglandin E1 (PGE1) treatment of 91 newborns with ductus dependent congenital heart disease (CHD). PGE1 efficacy, side-effects as well as the cardiovascular and respiratory profile of the patients were analysed. PGE1 doses > 0.02 microgram/kg per minute were used for only 5.3% of the total 23,656 h of treatment. The mean systolic blood pressures did not differ from the normal mean for patients with cyanotic CHD, while the diastolic values were lowered. Respiratory support was required only during 13.7% of the total treatment time. Apnoeas occurred in 21 (38%) of the 55 spontaneously breathing infants, who all had a cyanotic CHD. The incidence of apnoeas was lower during treatment with doses < 0.01 microgram/kg per minute. CONCLUSION: PGE1 can be successfully administered in lower doses than previously recommended. Especially high initial doses can be avoided and low maintenance doses allow long-term treatment without serious complications.

Left aortic arch and right descending aorta--interruption or severe coarctation of the aortic arch in the newborn.

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980 g, 3000 g and 3400 g respectively) with interruption of the aorta (n = 2) and severe coarctation (n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.

Low dose inhaled nitric oxide causing selective pulmonary vasodilation in child with idiopathic pulmonary hypertension.

Low dose (3 ppm) inhaled nitric oxide caused selective pulmonary vasodilation with improved systemic arterial pressure, cardiac index and arterial oxygenation in an infant with primary pulmonary hypertension. Methaemoglobin levels did not exceed 0.6%, and nitrogen dioxide concentrations remained within 0.05 ppm.