Predictive value of Cardiac Magnetic Resonance: new and old parameters in the natural history of repaired Tetralogy of Fallot.

BACKGROUND: Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. METHODS: 130 rTOF patients who underwent CMR (2006-2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. RESULTS: Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy. CONCLUSIONS: Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.

A PHACES syndrome unmasked by propranolol interruption in a tetralogy of Fallot patient: case report and extensive review on new indications of beta blockers.

Infantile hemangiomas (IHs) are the most common benign tumors of infancy and usually they don't require specific therapy. In 10-20% of cases IHs are able to generate complication and medical/surgical intervention is needed. For many decades standard treatment consisted in oral or intralesional corticosteroids until Leaute-Labreze and colleagues published the first report on the efficacy of propranolol for cutaneous infantile hemangiomas in 2008. IHs can be sometimes part of complex syndrome. Here we report the case of a patient with tetralogy of Fallot operated at 5 month of age who stopped propranolol treatment for hypoxic spells and unusually developed facial and subglottic IHs configuring the diagnosis of PHACES syndrome (posterior fossa brain malformations, hemangioma, arterial anomalies, cardiac defects and/or aortic coarctation, ocular anomalies and sternal defects). To our knowledge this is the first report in the international literature of a delayed appearance of an infantile hemangioma involving the skin and the airways (PHACES syndrome). The pathophysiological explanation relies on the mechanism of action of propranolol which seems to act initially with vasoconstriction, down-regulating proangiogenetic factors and inducing endothelial cell apoptosis. Many decades since their introduction ß-blockers are useful in a growing group of diseases. The pleiotropic effect of ß-adrenoceptors antagonists is not yet deeply understood, residing in neurohormonal regulation systems and angiogenesis and proving to be an effective treatment from cardiovascular to oncological illnesses.

Towards true unipolar ECG recording without the Wilson central terminal (preliminary results).

We present an innovative bio-potential front-end capable of recording true unipolar ECG leads for the first time without making use of the Wilson central terminal. In addition to the convenience in applications such as continuous monitoring and rapid diagnosis, the information in unipolar recordings may yield unique diagnostic information as it avoids the need to essentially subtract data or make use of the averaging effect imposed from the Wilson central terminal. The system also allows direct, real-time software calculation of signals corresponding to standard ECG leads which achieve correlations in excess of 92% with a gold standard ECG during a parallel in vivo recording. In addition, the implemented circuit is wideband (0.05-1000 Hz), compatible with standard (Ag/AgCl) bio-potential electrodes, and dry (paste-less) textile electrodes. The circuit is also low power, requiring less than 50 mW (when powered at 12 V) per standard ECG lead (two channels required). It is therefore well suited for wearable, long-term applications.

Noncoronary aortic cusp rupture in an adult patient with ventricular septal defect: echocardiographic diagnosis.

Aortic regurgitation is a common complication of ventricular septal defects. The most common mechanism is right or noncoronary cusp prolapse. Other mechanisms are right or noncoronary cusp fibrosis resulting in thickening and restricted motion of the leaflets or infective endocarditis leading to cusp perforation. We describe a case of subacute and severe aortic regurgitation due to noncoronary cusp prolapse resulting in the development of a large aneurysm and rupture of the noncoronary sinus of Valsalva into the right atrium.

Combined left pulmonary artery sling transposition and tracheal lobe resection through a median sternotomy: a case report.

INTRODUCTION: The aim of this paper was to report the management of a very rare malformative association of Left Pulmonary Artery (LPA) sling and Tracheal Lobe which is not still reported in scientific literature. MATERIALS AND METHODS: The Authors describe the clinical case of a 6 years old girl that was admitted for chronic respiratory symptoms associated with recurrent upper respiratory infections. The CT-scan with virtual bronchoscopy showed an ectopic bronchus arising from the right side of the upper third of the trachea and ending in an accessory pulmonary lobe, covered by normal pleura, located in the upper mediastinum. The tracheo-broncoscopy showed a stenotic tracheal lumen with complete cartilaginous ring with an evident vascular pulsation in the middle of stenotic tract. An angio-CT confirmed the vascular anomalies with the LPA which passes between the lower trachea and the accessory tracheal bronchus and the coexistence of a persistent left superior vena cava. The surgical approach, after the thoracoscopic exploration, was a double procedure through a median sternotomy with cardiopulmonary bypass has permitted to excise completely the tracheal lobe and to reimplant the left pulmonary artery into the main pulmonary artery. RESULTS: No more upper respiratory symptoms or pulmonary infections have been observed during the clinical and instrumental follow up. CONCLUSIONS: Current controversy in the management of Pulmonary Artery sling include surgical approach (median sternotomy versus left thoracotomy), use or non-use of cardiopulmonary bypass, and reimplantation versus translocation with distal tracheal resection. In our experience LPA reimplantation and tracheal lobe resection have been made easily and safely by the same sternotomy utilized for the cardio-pulmonary by-pass.