Dobutamine Stress Cardiac MRI in Pediatric Patients with Suspected Coronary Artery Disease.

Following current practice, pediatric patients with treated congenital coronary malformations or acquired coronary disease undergo Cardio-Pulmonary Exercise Test (CPET), stress Echocardiography and Electrocardiography (sEcho, sEKG), and Coronary Angiography (CA). Stress cMRI can assess cardiac function, myocardial viability, and stress/rest perfusion deficit-without radiation exposure, general anesthesia, and hospitalization-in a single non-invasive exam. The aim of our pilot study is to assess the feasibility and diagnostic accuracy of Dobutamine stress cMRI compared to the current procedures (sEcho, CPET, CA). The prospective study is focused on pediatric patients, at risk for or with previously diagnosed coronary artery disease: d-looped TGA after arterial Switch, Kawasaki disease, and anomalous origin of left coronary artery from pulmonary artery (ALCAPA) after coronary artery reimplantation. We have compared the results of MRI coronary angiography, and Dobutamine stress cMRI with traditional tests. All these diagnostic exams were acquired in a timeframe of 3 month, in a blinded fashion. All the 13 patients (age: 12 ± 2 years, median 12,7 y) recruited, completed the study without major adverse events. The mean heart rate-pressure product was 25,120 ± 5110 bpm x mm Hg. The target heart rate of 85% of the maximal theoretical was reached by 10 (77%) patients. The comparison between cardiac MRI coronarography versus the gold standard Coronary Angiography to identify the patency of the origin and the proximal pathway of the coronary arteries shows a sensitivity of 100% (confidence interval: 2,5-100%), specificity 92% (confidence interval: 64-100%). The stress test was well tolerated for the 77% of the patients and completed by the totality of patients (Table 3). Three patients (23%) had mild symptoms: nausea, vomiting, or general discomfort. In pediatric patients with a potential or definite diagnosis of coronary artery disease, stress cMRI combines an effective assessment of proximal coronary arteries anatomy with cardiac function, myocardial perfusion, and viability in a single examination. Stress cMRI can be proposed as alternative, standalone test.

Hybrid procedure of right ventricle outflow tract stenting in small infants with pulmonary atresia and ventricular septal defect: early and mid-term results from a single centre.

IntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow. METHODS: We retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes. RESULTS: Median age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12-103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months. CONCLUSIONS: We reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.

Early and Long-Term Results of Stent Implantation for Aortic Coarctation in Pediatric Patients Compared to Adolescents: A Single Center Experience.

Background. Stents have become the treatment of choice for native aortic coarctation in adults and adolescents, but in pediatric patients insufficient data are currently available to identify the best therapeutic option. Methods. To compare the outcomes of pediatric and adolescent patients, we retrospectively evaluated early and long-term results of stenting for aortic coarctation in 34 patients divided into 2 groups (A and B) composed, respectively, of 17 children (mean age 8.2 ± 2.3, weight ≤30 kg) and 17 adolescents (mean age 14.3 ± 1.7, weight >30 kg). Results. No significant differences in outcome were found between groups immediately after the procedure. In all of our patients, peak systolic gradient pressure significantly decreased after stenting from 43.7 ± 12 to 1.7 ± 3.1 mmHg in group A and from 39.4 ± 16.8 to 1.6 ± 3 in group B (p < 0.0001). We observed early and late adverse events in both groups: early femoral vessel injury or thrombosis was more frequent in younger patients, as well as restenosis due to vessel growth requiring stent redilatations, often complicated by stent fractures. Data from long-term follow-up showed that, in younger patients, stress-related hypertension was more frequent. Conclusions. The procedure was immediately safe and effective in both groups. Pediatric patients must be accurately selected before stenting because they could probably need reinterventions and stents could impact on their future therapeutic perspectives.

Tricuspid annular plane systolic excursion for the assessment of ventricular function in adults operated on with mustard procedure for complete transposition of the great arteries.

BACKGROUND: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients. METHODS: Patients were recruited from those evaluated at the adult congenital clinic of our department between 2010 and 2012. All patients who had an echocardiographic assessment within 6 months of their MRI examination were selected. Patients clinically unstable, not in sinus rhythm, with a prosthetic systemic atrioventricular valve, permanent pacemaker, or more than moderate systemic atrioventricular valve regurgitation were excluded. RESULTS: Eighteen Mustard-operated patients aged 22 ± 3.7 years were studied. The mean values of TAPSE and RVEF were 13.22 ± 1.7 mm and 49.7 ± 6%, respectively. TAPSE and RVEF were normal in 1 (5.5%) and 10 (55.5%) patients, respectively. Seventeen (94.4%) patients showed reduced TAPSE (12.9 ± 1.3 mm): RVEF was reduced in eight (47%) of these subjects, and normal in nine (53%). In patients with normal RVEF, both the MRI-RV end-diastolic and the MRI-RV end-systolic volumes were significantly lower than in patients with reduced RVEF. There were no other statistically significant differences between these patients. No correlation was found between TAPSE and both the MRI-RV end-diastolic and the end-systolic volumes. Globally, agreement between TAPSE and RVEF was slight (K = 0.09 ± 0.089). CONCLUSIONS: Our results indicate that in these patients TAPSE is not a useful measure of RV function.

Historia natural y evolución clínica de los pacientes con síndrome de la cimitarra «no corregido»: un estudio multicéntrico de la Sociedad Italiana de Cardiología Pediátrica / Natural history and clinical outcome of “Uncorrected” scimitar syndrome patients: a multicenter study of the Italian Society of Pediatric Cardiology

Introducción y objetivos. Analizar el estado clínico de los pacientes con síndrome de la cimitarra «no corregido» en un estudio multicéntrico italiano. Métodos. Se analizó la evolución natural del síndrome de la cimitarra en 44 individuos afectados de nueve centros de Italia. Resultados. La mediana de edad de diagnóstico fue 1,05 años (intervalo, 1 día-41 años). En total, 33 pacientes (75%) presentaban una forma aislada; en 11 (25%) había cardiopatías congénitas asociadas; 22 pacientes (50%) tenían síntomas en el momento del diagnóstico, que consistían en síntomas respiratorios (n = 20) e insuficiencia cardiaca congestiva (n = 6). Los pacientes con cardiopatías congénitas asociadas presentaban prevalencias de insuficiencia cardiaca congestiva (4 de 11 [36,4%] frente a 2 de 33 [6,1%]; p = 0,027) e hipertensión arterial pulmonar (7 de 11 [63,6%] frente a 2 de 33 [6,1%]; p = 0,027) superiores que los pacientes con formas aisladas del trastorno. A 10 pacientes (22,7%), se les practicó una corrección de los defectos cardiacos asociados, con lo que se dejó intacto el drenaje venoso pulmonar. La mediana de duración del seguimiento después del diagnóstico fue de 6,4 (0,2-27,5) años. Fallecieron 2 pacientes, ambos con defectos cardiacos asociados e hipertensión arterial pulmonar grave. De los 42 supervivientes, 39 (92,8%) estaban asintomáticos en la última visita de seguimiento; 3 pacientes continuaban refiriendo síntomas respiratorios. No se observaron diferencias entre las formas aisladas y las formas asociadas de la enfermedad. Conclusiones. En la mayoría de los pacientes, el síndrome de la cimitarra se manifestó en forma de una lesión aislada con evolución benigna. No obstante, cuando se asociaba a otros defectos cardiacos y a hipertensión arterial pulmonar, había aumento del riesgo de insuficiencia cardiaca congestiva y muerte. La corrección de los defectos cardiacos asociados (transformación de una forma «asociada» a una forma «aislada»), junto con la oclusión terapéutica de la irrigación arterial anómala del pulmón, proporcionó una evolución benigna comparable a la de las formas aisladas primarias (AU)

Introduction and objectives. To analyze the clinical status of patients with “uncorrected” scimitar syndrome in a multicenter Italian study. Methods. The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers). Results. The median age at diagnosis was 1.05 years (range, 1 day-41 years). Thirty-three patients (75%) had an isolated form; 11 patients (25%) had associated congenital heart diseases. Twenty-two patients (50%) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4%] vs 2 of 33 [6.1%]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6%] vs 2 of 33 [6.1%]; P=.027) than patients with isolated forms. Ten patients (22.7%) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8%) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease. Conclusions. In most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming “associated” into “isolated” forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms (AU)

Use of a telescopic system for transcatheter radiofrequency perforation and balloon valvotomy in infants with pulmonary atresia and intact ventricular septum.

BACKGROUND: Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum. METHODS AND RESULTS: In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A. CONCLUSION: In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients' exposure to procedure-related risks.

Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the italian society of pediatric cardiology.

INTRODUCTION AND OBJECTIVES: To analyze the clinical status of patients with "uncorrected" scimitar syndrome in a multicenter Italian study. METHODS: The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers). RESULTS: The median age at diagnosis was 1.05 years (range, 1 day-41 years). Thirty-three patients (75%) had an isolated form; 11 patients (25%) had associated congenital heart diseases. Twenty-two patients (50%) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4%] vs 2 of 33 [6.1%]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6%] vs 2 of 33 [6.1%]; P=.027) than patients with isolated forms. Ten patients (22.7%) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8%) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease. CONCLUSIONS: In most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming "associated" into "isolated" forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms.

Impact of prenatal diagnosis on outcome of pulmonary atresia and intact ventricular septum.

OBJECTIVES: To determine the impact of fetal echocardiography on the management of pregnancy and of newborns affected by pulmonary atresia and intact ventricular septum (PAIVS) and to evaluate the outcome of infants with and without prenatal diagnosis of PAIVS. METHODS: We searched our database for cases of PAIVS prenatally and postnatally diagnosed during the period January 1993-December 2009. Postnatal follow-up was available in all cases included in the study. Karyotyping and fluorescent in situ hybridization analysis for the DiGeorge critical region (22q11.2) were performed in all but one case. RESULTS: The study comprised 60 cases of PAIVS: 36 with (Group A) and 24 without (Group B) prenatal diagnosis. In Group A, there were two intrauterine deaths, six postnatal deaths (five early after birth) and one termination of pregnancy. In this group, radiofrequency (RF) perforation was successfully performed in 25 cases; 20/25 infants had a biventricular (BV) repair, without further operation in 13 of them. No patient of Group B died. In this group, RF perforation was successfully performed in 22 cases; 20/22 had a BV repair without further procedure in 15 of them. CONCLUSIONS: Prenatal diagnosis of PAIVS allows a reliable prognosis of severity and planning of proper surgical repair strategies. Fetuses that are prenatally diagnosed present a more severe spectrum of the disease; for the cases capable of getting through the neonatal period, the mortality rate and the need for further intervention were not significantly different when compared with babies with only postnatal diagnosis.

Selective pulmonary artery embolization in two patients with single ventricle and acquired pulmonary vein occlusion.

Acquired pulmonary vein occlusion is a rare complication of surgical correction of complex congenital heart diseases. Associated vascular remodeling of arterial and venous vessels includes medial thickening, intimal fibrosis, and progressive development of aorto-pulmonary collaterals. The consequent pulmonary perfusion changes impact more importantly on a low pressure circulation such as the Fontan circuit. The management of these patients is still controversial. We describe this condition in two patients with single ventricle who underwent staged cavopulmonary connection. In both cases, we found acquired occlusion of one pulmonary vein, poor antegrade flow in the involved hypoplastic pulmonary arteries due to a widespread thin collateral circulation producing a backward washout of unopacified blood flow with competitive mechanism. Percutaneous embolization of a segmental pulmonary artery was performed with restoration of a more homogeneous perfusion of both main pulmonary arteries. Extracardiac total cavopulmonary connection was successfully performed a few months later in both patients.

Prenatal diagnosis and outcome of isolated vascular rings.

The purpose of this study was to describe our experience in the ultrasound visualization and management of prenatally diagnosed isolated vascular rings. From January 2002 to December 2007, a total of 19 fetuses had a sonographic diagnosis of isolated vascular rings in 2 reference centers at a mean gestational age of 23 weeks. There were 8 cases of left aortic arch with aberrant right subclavian artery, 5 cases of double aortic arch, and 6 cases of right aortic arch with aberrant left subclavian artery. Two fetuses had associated trisomy 21 and 1 had a 22q11 microdeletion. Parents chose to terminate the pregnancy in all cases. Four patients successfully underwent surgical correction, and in 1 patient, tracheoplasty was also performed. In conclusion, isolated vascular rings can be accurately diagnosed prenatally by using the "3-vessel and trachea view" and "supra-aortic-branch view" that allow detection of vascular structures running around the trachea. Karyotyping and prenatal testing for 22q11 microdeletions should be offered to all parents. Affected children should undergo surgical correction as soon as symptoms of tracheal compression appear, avoiding tracheomalacia. Associated congenital tracheal stenosis should be excluded before surgery.

Periodontal disease and coronary heart disease: an epidemiological and microbiological study.

AIMS: This is an investigation on the association between periodontal disease and an increased risk of coronary heart disease; the main hypothesis is that periodontal infections may increase the systemic inflammatory burden of the host above a threshold that may favour the atherogenic processes. MATERIALS AND METHODS: Case-control study with 27 cases, cardiologically affected, and 15 healthy controls. Patients underwent a complete periodontal probing. Periodontal conditions were compared between cases and controls to assess the mentioned association and to search for periodontal conditions related to the increased coronary risk. The presence and prevalence of periodontal pathogens was assessed in crevicular fluid samples. RESULTS: The overall periodontal conditions resulted worse in the test group. In particular periodontal conditions such as the presence of deep pockets (probing depth >6 mm) and the loss of more than 12 teeth might represent indicators of a strongly increased risk of cardiological disease and microbiological investigations confirmed these findings; Prevotella gingivalis was the most common bacteria. CONCLUSION: This study supports the existence of an epidemiologic association between periodontal disease and coronary heart disease and confirms previous data present in the literature. Two periodontal parameters, deep pockets and number of missing teeth, seem to be important risk factors for cardiovascular diseases.