Coronary unroofing does not fits all anomalous aortic origin of coronary arteries.

INTRODUCTION: Anomalous aortic origin of coronary artery (AAOCA) is the second leading cause of sudden cardiac death in children and young adults. Intramural-interarterial course is the most frequent anatomic variation and coronary unroofing is widest adopted for surgical management. Symptoms recurrence is described regardless of the technique used. This study aims to describe how an anatomic patient-centered approach aimed to restore a normal coronary artery take-off is associated with symptoms resolution. METHODS: From 2008 to 2021, 25 patients were operated on for an AAOCA at a median age of 20 years. Nineteen patients had a right AAOCA and six had left AAOCA. Intramural course was present in 18 patients. Seventy-six percent were symptomatic. No episodes of aborted sudden cardiac death before surgery was described in the population. Surgical technique used were coronary unroofing in 18 patients, coronary neo-ostioplasty in 3, coronary Reimplantation in 3, and main pulmonary artery re-location in 1. RESULTS: No hospital mortality or reoperation was observed in our experience as well as major complications related to surgery. Mean hospital length of stay was 8.5 days. None of patients reported symptoms recurrence at follow-up. Young athletes returned to play competitive sport. Postoperative computed tomography scan evaluation showed a general improvement of the take-off angle. CONCLUSIONS: AAOCA requires a patient anatomic-based surgical management. There is not a single surgical technique that can fits all anatomic subtype of AAOCA. Surgical techniques may be selected on the base of the preoperative images and intraoperative findings. In our experience, this policy is associated with no symptoms recurrence.

Serial cardiac magnetic resonance imaging for guidance of therapy management in patients treated with anakinra due to recurrent pericarditis.

Aims: To determine the utility of serial cardiac magnetic resonance (CMR) imaging for guidance of therapy management in patients treated with anakinra due to recurrent pericarditis (RP), compared with C-reactive protein (CRP) assay alone. Methods and results: In 2018-21, we enrolled 18 (14.5 ± 1.8 years old, 72% males) consecutive RP patients treated with anakinra (100 mg/day in patients ≥ 18 years old; 2 mg/kg/day < 18 years old) due to RP corticosteroid-dependent or not responsive to colchicine or non-steroidal anti-inflammatory drugs. After hospitalization, they were 1:1 randomized to CMR [no pericardial oedema and/or late gadolinium enchantment (LGE)] or CRP (<0.6 mg/dL). Tests were repeated every 3 months until negative to halve the anakinra dosage and cessation. The idiopathic aetiology was the most prevalent (n = 8, 44%), followed by post-pericardiotomy (n = 6, 33%). After a median treatment period of 8.7 ± 3.6 months, CRP-guided RP patients experienced more recurrences than CMR-guided ones (6 vs. 1, P = 0.016), with the worst prognosis in terms of recurrences (log-rank, P = 0.025) and significantly increased time of treatment (12.7 ± 2 vs. 16.1 ± 3.4 months, P = 0.019). In a multivariable exploratory Cox regression model, the number of previous recurrences and the idiopathic aetiology were independent predictors of RP during the anakinra treatment. New recurrences were subsequently directed to CMR imaging, and therapy was modified according to the LGE/oedema trend. After 1-year follow-up, no further recurrence was detected. Conclusion: Among patients with RP and treated with anakinra, serial CMR imaging of the pericardium can be utilized as an imaging biomarker, more informative for therapy duration than the solely CRP assessment. ClinicalTrialsgov Identifier: NCT06071156.

Foetal ductus arteriosus constriction unrelated to non-steroidal anti-Inflammatory drugs: a case report and literature review.

Foetal ductus arteriosus (DA) constriction can be found in complex foetal heart malformations, but rarely as an isolated defect. Although many cases of DA constriction are usually related to Non-steroidal Anti-Inflammatory Drugs (NSAIDs) maternal intake, other causes remain without an established aetiology and are referred to as idiopathic. Recently, a wide range of risks factors or substances (polyphenol-rich foods intake, naphazoline, fluoxetine, caffeine and pesticides) showed a definitive effect upon the pathway of inflammation, causing DA constriction. We report a case of a premature DA constriction in a woman whose possible risk factor was identified in her maternal occupational exposure to solvents and a comprehensive literature review of 176 cases of NSAID-unrelated DA constriction. A 30-year-old Asian woman was referred to our institution at 33 gestational weeks and 0 days because of suspicion of premature DA constriction. The woman had no history of medication intake, including NSAIDs, alcohol, tobacco or polyphenol-rich-food consumption during pregnancy. A detailed foetal echocardiography revealed a normal cardiac anatomy with hypertrophic, hypokinetic and a dilated right ventricle due to right pressure overload, holosystolic tricuspid regurgitation, and, at the level of the DA, high systolic and diastolic velocities, indicating premature ductal restriction. The right outflow showed dilatation of the pulmonary artery with narrow DA. An urgent caesarean section was performed at 33 gestational weeks and 4 days due to worsening of DA PI and signs of right pressure overload, despite the interruption of exposure to solvents. We assume a relationship exists between premature DA constriction and a maternal occupational exposure to solvents. This hypothesis is reinforced by the presence of associated foetal malformations in in two of the patient's children. Further research is needed to confirm the role of exposure to solvents and toxic chemicals in the pathogenesis of DA constriction, also with experimental animal models.KEY MESSAGESMany cases of DA constriction are usually related to Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) maternal intake.A wide range of risks factors or substances (polyphenol-rich foods intake, naphazoline, fluoxetine, caffeine and pesticides) can cause foetal DA constriction.Further investigation are needed to confirm the role of maternal exposure to solvents in the pathogenesis of DA constriction.

Echocardiographic screening for the anomalous aortic origin of coronary arteries.

AIMS: We sought to determine the diagnostic performance, clinical profiles and outcomes of anomalous aortic origin of coronary arteries (AAOCA) using a standardised echocardiographic approach in young adults and athletes. METHODS: In 2015-2019, we screened 5998 outpatients (age 16 years (Q1-Q3: 11, 36)), referred for routine echocardiography, using four specific echocardiographic windows: parasternal short/long axis and apical 4/5-chambers view. Coronary CT confirmed AAOCA. For the performance analysis, 300 coronary-CT scans were available; two independent and double-blinded physicians retrospectively reviewed echocardiographic images. RESULTS: A total of 47 AAOCA was diagnosed; the overall prevalence was 0.0078%. Over 5 years, we found a significant increment of AAOCA diagnostic rate (P for trend=0.002). Syncope (n=17/47) and palpitations (n=6/47) were prevalent symptoms. All patients suspended sports activity at the diagnosis. Twenty-seven patients underwent surgery, while 20 underwent a conservative medical treatment. All patients are alive at a median follow-up of 3±1.6 years; only surgical repairs restarted their activity. Our method showed better sensitivity than traditional short-axis evaluation: 93% vs 83%, p=0.0030 (AUC 0.96 (95% CI 0.92, 0.99) and AUC 0.89 (95% CI 0.83, 0.95), respectively), with a good interobserver agreement (95%, k=0.83, p<0.001). CONCLUSIONS: The application of a standardised echocardiographic approach for AAOCA detection led to a significantly increased rate of identified anomalies. This approach demonstrated higher sensitivity than the traditional echocardiographic assessment. Implementing this protocol in clinical practice may help improve the AAOCA diagnosis in young adults and athletes. TRIAL REGISTRATION NUMBER: NCT04224090.

Echocardiographic changes and quality of life after surgical unroofing of myocardial bridges.

OBJECTIVE: To examine the echocardiographic changes and quality of life (QoL) after surgical unroofing of myocardial bridges (MBs) involving the left anterior descending (LAD) coronary artery compared with optimal medical therapy (OMT). METHODS: Twenty-two patients (median age, 24 years; interquartile range, 16-40 years) with MBs of the LAD artery and exertional angina from 2018 to 2019 were retrospectively analyzed. Twelve patients underwent OMT and 10 underwent surgery. Both groups underwent clinical and echocardiographic examinations during hospitalization and follow-up (mean, 1.0 ± 0.8 years). QoL was assessed with the Seattle Angina Questionnaire, short version (SAQ-7). RESULTS: Surgery resulted in significantly better QoL than OMT, with a significant improvement in left ventricular global longitudinal strain (GLS) [mean (standard error): 19% (0.19) to 22% (0.34) and 19% (0.15) to 20% (0.24), respectively; delta-change (delta-GLS) of 0.15 vs. 0.067]. In the univariate and multivariable analyses, delta-GLS was positively correlated with the SAQ-7 score and MB length (rho = 0.64 and 0.71, respectively), with a significant interaction between MB length and surgical treatment (beta coefficient, 1.95; 95% confidence interval, 0.14-3.77). CONCLUSIONS: MB unroofing surgery provided benefits in terms of QoL and left ventricular GLS improvement compared with 1 year of OMT.

[Cardiologists and mucopolysaccharidoses. Recommendations of GICEM (Cardiology Experts on Metabolic Disease Italian Group) for diagnosis, follow-up and cardiological management]. / Il cardiologo e le mucopolisaccaridosi. Raccomandazioni del GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) su diagnosi, follow-up e management cardiologico.

Mucopolysaccharidoses (MPS) represent a group of rare lysosomal storage disorders, with a heterogeneous clinical presentation in terms of inheritance (autosomal and X-linked recessive), age of onset (infants, children, and adults), systemic and cardiac manifestations (mild to severe disease forms). Evidence-based recommendations on the diagnosis and management of cardiovascular disease in MPS are scarce. GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) is a group of cardiologists, cardiac surgeons and pediatricians with a specific expertise in metabolic diseases including MPS. In this paper, we report our experience and recommendations on the diagnosis and management of cardiovascular aspects in MPS, with a tailored approach based on current evidence, and taking into account MPS phenotype (particularly, I, II, IVa, VI), age at presentation, and severity of systemic and cardiac manifestations.

Pulmonary artery banding: when is the use of a telemetrically adjustable device indicated?

Proponents of a telemetrically adjustable pulmonary artery band (PAB) device have cited simplified postoperative management and shortened length of stay as advantages associated with that technology. This report concerns a recent experience with both conventional pulmonary artery banding (conv-PAB) and the telemetrically adjustable PAB FloWatch (FW-PAB). From January 2005 through December 2008, 19 consecutive infants underwent either conv-PAB (8 patients, mean age 3.5 months, mean weight 4.1 kg) or FW-PAB (11 patients, mean age 2.6 months, mean weight 3.1 kg). Indications for PAB were left ventricular retraining (1 patient in FW-PAB), palliation prior to biventricular repair (7 patients in conv-PAB and 10 in FW-PAB group), and staged univentricular repair (1 patient in conv-PAB). In-hospital mortality was 0%. In the FW-PAB group, 1 FloWatch device was removed because of hemodynamic compromise related to the bulk of the device. There were no major complications in the conv-PAB group and no differences between groups with respect to postoperative ventilation time or length of stay in the intensive care unit or in hospital. In the FW-PAB group, a mean of 3.1 ± 1.7 regulations per patient were undertaken. Of the regulations, 85% (29/34) were adjustments to tighten the device, and 15% (5/34) were to loosen it. During follow-up, 8 patients underwent intracardiac repair and pulmonary artery debanding: 4 in the conv-PAB group and 4 in the FW-PAB group. The course of patients in both groups after PAB were similar. Major differences in length of stay and resource utilization were not apparent.

Valve repair in congenital aortic valve abnormalities.

Many surgical techniques have been described either to repair and to replace the aortic valve. Among the paediatric population the potential for growth has to be preserved and valve reconstruction is therefore of great importance. In the last two years 25 consecutive patients, mean age 8.6 years, with aortic valve disease, underwent aortic valve repair for aortic regurgitation (AR) (10 patients), aortic valve stenosis (5 patients) or mixed lesion (10 patients). None of the patients died neither during hospitalization nor at follow-up (median 9.25 months). In the aortic stenosis (AS) group, one patient required reoperation (re-repair). None of the patients in the AR group developed more than mild AS and mild AR during follow-up. Nine out of 10 patients of the mixed lesion group had no or trivial AR at the follow-up. Left ventricular dimension decreased in all patients after repair. With a better understanding of the causes of AS or AR and the adoption of different techniques, often used in multiple association, we believe that aortic valve repair can be achieved in most patients with a normal left ventricular outflow tract.

Edge-to-edge technique: is it also useful in children?

OBJECTIVE: Mitral valve prolapse is a rare condition and represents a challenge for cardiac surgeons. Many techniques have been described to treat the wide spectrum of abnormalities causing mitral valve regurgitation but the low incidence of these abnormalities is the cause of the relatively poor experience of many institutions. METHODS: From April 2005 to September 2006, five patients underwent isolated mitral valve repair with the edge-to-edge technique for anterior or bileaflet prolapse. Three patients also had a Gore-Tex strip to reinforce the posterior annulus, whereas two had a 26-size soft incomplete ring annuloplasty. RESULTS: There was no death and none of the patients required reoperation. None of the patients had mitral regurgitation more than 1+ at a mean follow-up of 30 months. None of the patients had mitral stenosis or any valve-related event. CONCLUSION: The edge-to-edge technique is a well tolerated and useful technique for the anterior or bileaflet prolapse of the mitral valve. Although many techniques have been described with good results, in many anatomical settings and in small patients, edge-to-edge is a good option and might not interfere with the growth of the valve. Early results are encouraging but a longer follow-up is needed to evaluate the long-term prognosis.

Reimplantation of an anomalous coronary artery arising from the pulmonary artery.

A case of anomalous origin of the left coronary artery from the pulmonary artery in a patient with the origin of the coronary opposite to the aorta is reported. Between many surgical options we conclude to reestablish a double coronary system reconnecting the coronary through a conduit created with a pulmonary wall baffle and an autologous pericardial patch.