Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA): Echocardiographic diagnosis in a critically ill newborn.

ARCAPA echocardiographic diagnosis. A network of small multiple coronary vessels, a systo-diastolic jet of flow into the pulmonary artery and a retrograde inverted «blue¼ flow into the RCA are the key signs. CT-scan confirms the diagnosis .

Pediatric airway compression in aortic arch malformations: a multidisciplinary approach.

Background: Aortic arch malformations (AAMs) should be suspected in the presence of persistent respiratory symptoms despite medical treatment or feeding problems at the pediatric age. Aim: We report a descriptive cohort of patients with AAMs and the local management protocol applied. Methods: A total of 59 patients with AAM were retrospectively reviewed. Three groups were identified: double aortic arch (DAA), group 1; complete vascular ring (non-DAA), group 2; and anomalous origin of the innominate artery (IA), group 3. Results: Prenatal diagnosis was available for 62.7% of the patients. In all, 49.2% of children were symptomatic. There was a significantly different prevalence of respiratory symptoms within the three groups: 73.7% in group 1, 24.2% in group 2, and 100% in group 3 (p-value: <0.001). Surgery was considered in the presence of symptoms in patients with DAA and in those with reduction of the tracheal section area greater than 50%. A total of 52.5% of the patients underwent surgical repair (median age 6 months). The median follow-up interval was 21.9 months. Respiratory symptoms improved in most symptomatic patients. Conclusions: No specific protocols are available for the management of patients with AAMs. Conservative treatment seems to be reasonable for asymptomatic patients or those with airway stenosis less than 50%. A close follow-up is necessary to identify early patients who become symptomatic.

Prenatal prediction of Shone's complex. The role of the degree of ventricular disproportion and speckle-tracking analysis.

OBJECTIVES: Shone's complex (SC) is characterized by sequential obstructions of left ventricular (LV) inflow and outflow. It can be associated with poor long-term prognosis when compared to Simple-Aortic Coarctation (S-CoA). We aimed to assess whether the degree of ventricular disproportion and 2D-speckle-tracking echocardiography (2D-STE) could improve the accuracy of prenatal prediction of SC. METHODS: 75 consecutive fetuses were retrospectively enrolled from January 2010 to June 2021. Fetuses were divided into 4 groups (Group 1: SC; Group 2: S-CoA; Group 3: False Positive-Coarctation of the Aorta [FP-CoA]; group 4: controls). Comparisons for echocardiographic measures and myocardial deformation indices were performed. A receiver operating characteristic (ROC) analysis was performed on the MV/TV (mitral valve/tricuspid valve ratio) and LV GLS (global longitudinal strain) values to identify cut-offs to separate group 1 and 2 fetuses. RESULTS: SC fetuses showed a significant reduction in MV/TV when compared to S-CoA and FP-CoA fetuses (p<0.001). LV GLS in SC fetuses was significantly reduced compared to S-CoA fetuses (-13.3 ± 2.1% vs. -17.0 ± 2.2%, p=0.001). A cut-off value of 0.59 for MV/TV and -15.35% for LV GLS yielded a sensitivity of 76 and 82% and a specificity of 71 and 83% respectively in separating SC vs. S-CoA fetuses. CONCLUSIONS: SC fetuses showed a more severe degree of ventricular disproportion and a lower LV GLS compared to S-CoA, FP-CoA and control fetuses. MV/TV and GLS are both predictors of SC. These findings may improve the quality of prenatal parental counselling.

Echocardiographic diagnosis of a large coronary artery fistula in a newborn.

Coronary artery fistula (CAF) is a rare cause of heart murmur in newborns, with a very heterogeneous clinical presentation and unpredictable natural history. We described the case of a large neonatal CAF in an asymptomatic newborn. The patient did not show any symptoms of heart failure during a three-month follow-up. In this case, echocardiography was as accurate as CT scan in describing the anatomy and the course of CAF.

The Role of Lung Function Testing in Newborn Infants With Congenital Thoracic Arterial Anomalies.

Introduction: Congenital thoracic arterial anomalies (CTAAs), such as complete or incomplete vascular rings, pulmonary artery sling, and innominate artery compression syndrome, may cause severe tracheomalacia and upper airway obstruction. An obstructive ventilatory pattern at lung function testing (LFT) has been suggested in the presence of CTAA. The severity of obstruction may be evaluated by LFT. Little is known about the use of LFT in newborn infants with CTAA. The aim of our study is to evaluate the role of LFT in CTAA diagnosis. Methods: This is a retrospective study, conducted between February 2016 and July 2020. All CTAA cases for whom LFT was performed preoperatively were considered for inclusion. Tidal volume (Vt), respiratory rate, and the ratio of time to reach the peak tidal expiratory flow over total expiratory time (tPTEF/tE) were assessed and compared to existing normative data. Demographics and CTAA characteristics were also collected. Results: Thirty cases were included. All infants with CTAA showed a significantly reduced Vt and tPTEF/tE, compared to existing normative data suggesting an obstructive pattern. No significant differences were found for LFT between cases with a tracheal obstruction <50% compared to those with tracheal obstruction ≥50%, or between cases with and without symptoms. Sixteen infants (53.3%) had respiratory symptoms related to CTAA. Of these, only two cases had also dysphagia. Conclusion: LFT values were significantly reduced in cases with CTAA before surgery. LFT represents a potential feasible and non-invasive useful tool to guide diagnosis in the suspect of CTAA.

Should pre-operative left atrial volume receive more consideration in patients with degenerative mitral valve disease undergoing mitral valve surgery?

BACKGROUND: Severe primary mitral regurgitation (MR) carries a significant incidence of mortality and morbidity. Though a number of prognostic factors have been identified, the best timing for mitral valve repair is still debated. We assessed the role of Left Atrial Volume Indexed (LAVI) as predictor of adverse events after mitral valve surgery. METHODS: 134 patients with severe MR were studied with a follow-up of 42±16months. Endpoints were Post-Operative Atrial Fibrillation (POAF), atrial and ventricular remodeling (LARR/LVRR) and correlation with outcome. POAF was defined as AF occurring within 2weeks and late AF (LAF) more than 2weeks after surgery. LARR was defined as LAVI reduction ≥15% and LVRR as any reduction of ventricular mass after surgery. RESULTS: Forty-one patients experienced POAF, 26 had LAF. Pre-operative LAVI was an independent risk factor for POAF (OR 1.03, CI [1.00-1.06], p=0.01), LAF (OR 1.03, CI [1.00-1.06], p=0.02), LARR and LVRR (OR 1.04, CI [1.01-1.07], p=0.002, respectively). LARR was found in 75 patients, while LVRR in 111. Patients with heart remodeling had less incidence of LAF and cardiac adverse events, better diastolic function and improved their NYHA class after surgery. CONCLUSIONS: LAVI should be given more weight into decision making for patients with MR as it predicts POAF and LAF and reverse atrial and ventricular remodeling, both associated to long-term outcome.

ECG is an inefficient screening-tool for left ventricular hypertrophy in normotensive African children population.

BACKGROUND: Left ventricular hypertrophy (LVH) is a marker of pediatric hypertension and predicts development of cardiovascular events. Electrocardiography (ECG) screening is used in pediatrics to detect LVH thanks to major accessibility, reproducibility and easy to use compared to transthoracic echocardiography (TTE), that remains the standard technique. Several diseases were previously investigated, but no data exists regarding our study population. The aim of our study was to evaluate the relationship between electrocardiographic and echocardiographic criteria of LVH in normotensive African children. METHODS: We studied 313 children (mean age 7,8 ± 3 yo), in north-Madagascar. They underwent ECG and TTE. Sokolow-Lyon index was calculated to identify ECG-LVH (>35 mm). Left ventricle mass (LVM) with TTE was calculated and indexed by height(2.7) (LVMI(2.7)) and weight (LVMI(w)). We report the prevalence of TTE-LVH using three methods: (1) calculating percentiles age- and sex- specific with values >95th percentile identifying LVH; (2) LVMI(2.7) >51 g/m(2.7); (3) LVMI(w) >3.4 g/weight. RESULTS: 40 (13%) children showed LVMI values >95th percentile, 24 children (8%) an LVMI(2.7) >51 g/m(2.7) while 19 children (6%) an LVMI(w) >3.4 g/kg. LVH-ECG by Sokolow-Lyon index was present in five, three and three children respectively, with poor values of sensitivity (ranging from 13 to 16%), positive predictive value (from 11 to 18%) and high values of specificity (up to 92%). The effects of anthropometrics parameters on Sokolow-Lyon were analyzed and showed poor correlation. CONCLUSION: ECG is a poor screening test for detecting LVH in children. In clinical practice, TTE remains the only tool to be used to exclude LVH.

Congenital Absence of Left Atrial Appendage in a Patient with Intracranial Hemorrhage.

BACKGROUND: Intracranial hemorrhage is the most serious complication of anticoagulant therapy and is itself an absolute contraindication to further treatment. CASE REPORT: We present the case of a 78-year-old patient with permanent atrial fibrillation and previous intracranial hemorrhage during oral anticoagulation therapy, who was a candidate for percutaneous closure of the left atrial appendage. Transesophageal echocardiography and computed tomography showed absence of the left atrial appendage. The patient continued with single antiplatelet therapy. CONCLUSIONS: Absence of the left atrial appendage is a very rare congenital condition usually found in patients scheduled for cardiovascular procedures and without clinical significance. The risk of thromboembolism is reasonably low but unknown.

Percutaneous Treatment of Recurrent In-Stent Restenosis of Carotid Artery Stenting: A Case Report and State-of-the-Art Review.

BACKGROUND: Restenosis after carotid artery stenting (CAS) is a poorly described phenomenon. Studies have reported a variable incidence ranging from 4% to 19.7% at 1 year of follow-up. Doppler Ultrasound (DUS) is now routinely used in the follow-up after CAS and endarterectomy with optimal accuracy in detecting significant restenosis, compared to digital subtraction angiography (DSA). CASE REPORT: We reported the case of a 76-year-old patient with evidence of recurrent severe in-stent restenosis (ISR) of the left internal carotid artery (ICA). In April 2007, due to evidence at DUS of severe left ICA disease, the patient underwent CAS. In January 2009, due to DUS evidence of severe ISR, the patient underwent balloon angioplasty. In September 2011, DUS showed a severe ISR with a peak systolic velocity (PSV) of 436 cm/s; in June 2012 angiography showed a sub-expanded stent in the middle medial side with severe ISR (70%). Multiple inflations were performed and a slight residual sub-expansion of the lateral side of the stent was observed. Post-procedural DUS showed a reduction of PSV to 283 cm/s and 266 cm/s at 1-month follow-up. An increasing value (322 cm/s) was noticed at 3-month follow-up DUS, while at 6-month follow-up DUS showed an important increase to 483 cm/s. Strict follow-up was adopted because of the patient's refusal of further treatment. CONCLUSIONS: Criteria for diagnosis of restenosis are not well established. The optimal treatment is still debated and no indications have been established, due to the lack of sufficient data. Approaches to ISR include percutaneous transluminal angioplasty, cutting-balloon angioplasty (CB-PTA), stenting, and drug-eluting balloon (DEB) angioplasty. Several studies indicate that endovascular treatment, including balloon angioplasty and (CB-PTA) alone or in conjunction with stenting, is the preferred strategy.