Narrative review of assessing the surgical options for double outlet right ventricle.

The individualized surgical approach in individuals with both arterial trunks arising from the morphologically right ventricle is dictated by the extreme morphological variability encountered in this setting, with each patient being unique. An individualized surgical approach has been designed to take account of the morphological variations, identifying the anatomy with the preoperative three-dimensional CT scan reconstruction. The key features have been considered the distance between tricuspid and pulmonary valves, the size and location of the interventricular communication, and the relationship between the outflow tracts. The surgical approach is tailored, whenever feasible, to create a connection between left ventricle and aorta, but primarily to achieve biventricular repair. Account has been taken of all available surgical options already reported in the literature, identifying the most suitable to provide the best outcomes for each unique morphology. To date, meaningful comparison between different reported surgical series has been difficult because of the marked variation of individual intracardiac morphology, and the lack of reports of specific surgical approaches for well-categorized groups of patients. Our approach, being tailored to the individual cardiac morphology, can be offered to any patient with this ventriculo-arterial connection. Given the difficulties of diagnosis, and the multiple therapeutic indications, very close collaboration between cardiologists and surgeons is indispensable for further progress in the understanding and management of this complex congenital cardiac lesion.

Making the decision to donate eyes: Perspectives from the families of the deceased in Madurai, India.

PURPOSE: To identify factors affecting family members' decision whether to donate eye organs. METHODS: A community-based case-control study based on in-home interviews with families of deceased individuals who had or had not donated eye organs, in Madurai district, Tamil Nadu, India. Data collected were knowledge and awareness of eye donations, whether the deceased individual had expressed or pledged willingness to donate, and family members' attitudes and willingness to donate their own eye organs. RESULTS: Seventy-six families of donors and 256 families of non-donors completed the survey. Multivariable analysis showed that the following variables were significantly associated with a donation: age, whether the deceased had registered for eye donation, pre-expressed willingness of deceased to donate, whether family members personally know beneficiaries of eye donations, and higher score on a scale evaluating knowledge and awareness about eye donation. The majority of donors' families (71%) had been encouraged by someone to donate. Among non-donor families, a substantially larger fraction (52.8%) indicated they would have donated had someone reminded or encouraged them to do so, in comparison with those who indicated lack of awareness or knowledge (14.5%). CONCLUSION: Community programs are likely to be effective if they encourage individuals to pledge their eyes or express their willingness to donate their eyes to family members in advance of death; they increase public awareness of the value of eye donation. A friend, family member, neighbor or counselor approaching bereaved families and having a dialogue about eye donation would substantially increase the probability of a decision to donate.

Aorto-Right Ventricular Tunnel in Transposition of the Great Arteries.

Aorto-ventricular tunnel is an extremely rare congenital heart defect, consisting of failure of attachment of an aortic leaflet along the semilunar hinge. In all published reports the leaflet involved was either the right coronary leaflet, most frequently, or the left coronary leaflet, in most of the cases opening toward the left ventricle, with only one-eighth of the reported cases communicating with the right ventricle. Treatment of the aorto-ventricular tunnel has been anecdotally reported by interventional closure with a device and more frequently with surgical approach, either as an isolated malformation or as associated lesions. To the best of our knowledge, the presence of an aorto-ventricular tunnel of the non-adjacent aortic leaflet in transposition of the great arteries has never been reported. We have observed an aorto-ventricular tunnel involving the non-adjacent leaflet of the aortic root, which after arterial switch became the pulmonary root. The patient presented 18 years after the arterial switch with progressive dilatation of the right ventricle due to severe degree of pulmonary valve regurgitation, confirmed by echocardiography and cardiac MRI. Indication for surgery was given with the plan for a pulmonary valve implantation. Because of the intra-operative finding of disconnection of the anterior leaflet of the pulmonary valve (former aortic valve) along the semilunar hinge, the surgical plan was modified and the anterior leaflet was attached to the valve annulus, with subsequent plasty in correspondence with the right and left commissurae to reduce the size of the dilated annulus to normal diameter. The post-operative course was uneventful, with extubation after few hours and discharge 4 days after surgery, with echocardiography showing trivial degree of pulmonary valve regurgitation. The patient remains in good conditions 6 months after surgery.

Timely Pulmonary Valve Replacement May Allow Preservation of Left Ventricular Circumferential Strain in Patients with Tetralogy of Fallot.

INTRODUCTION: Patients with Tetralogy of Fallot (TOF) and pulmonary insufficiency and a dilated right ventricle (RV) may suffer from a reduction in left ventricular (LV) performance. It is not clear whether timely pulmonary valve replacement (PVR) preserves LV mechanics. METHODS: Ten TOF patients who underwent PVR were identified from hospital records, and pre- and postoperative cardiac magnetic resonance images were post-processed with a semi-automatic tissue tracking software. LV circumferential strain, time to peak strain, and torsion were compared before and after PVR. A control group of 10 age-matched normal volunteers was assessed as a comparison. RESULTS: LV circumferential strain did not change before vs. after PVR (basal -18.3 ± 3.7 vs. -20.5 ± 3%, p = 0.082; mid-ventricular -18.4 ± 3.6 vs. -19.1 ± 2%, p = 0.571; apical -22.7 ± 5.2 vs. -22.1 ± 4%; p = 0.703). There was also no difference seen between the baseline strain and normal controls (control basal -18.2 ± 3.3%, p = 0.937; mid -18 ± 3.2%, p = 0.798; apex -24.1 ± 5%, p = 0.552). LV torsion remained unchanged from baseline to post PVR [systolic 2.75 (1.23-9.51) °/cm vs. 2.3 ± 1.2°/cm, p = 0.285; maximum 5.5 ± 3.5°/cm vs. 2.34 (1.37-8.07) °/cm, p = 0.083]. There was no difference in time to measured peak LV circumferential strain before vs. after PVR (basal 0.44 ± 0.1 vs. 0.43 ± 0.05, p = 0.912; mid-ventricular 0.42 ± 0.08 vs. 0.38 ± 0.06, p = 0.186; apical 0.40 ± 0.08 vs. 0.40 ± 0.06, p = 0.995). At the same time, pulmonary regurgitation and RV end-diastolic and end-systolic volume indices decreased and LV end-diastolic volume increased after PVR. RV and LV ejection fractions remained constant. CONCLUSION: PVR allows for favorable remodeling of both ventricular volumes for TOF patients with significant pulmonary regurgitation. In this cohort, LV myocardial functional parameters such as circumferential strain, time to peak strain, and LV torsion were normal at baseline and remain unchanged after PVR.

Virtual 3D Modeling of Airways in Congenital Heart Defects.

The involvement of the airway is not uncommon in the presence of complex cardiovascular malformations. In these cases, a careful inspection of the relationship between the airway and the vasculature is paramount to plan the surgical procedure. Three-dimensional printing enhanced the visualization of the cardiovascular structure. Unfortunately, IT does not allow to remove selected anatomy to improve the visualization of the surrounding ones. Computerized modeling has the potential to fill this gap by allowing a dynamic handling of different anatomies, increasing the exposure of vessels or bronchi to show their relationship. We started to use this technique to plan the surgical repair in these complex cases where the airway is affected. This technique is routinely used in our Institution as an additional tool in the presurgical assessment. We report four cases in which the airways were compressed by vascular structures - ascending aorta in one, left pulmonary artery sling in one, patent ductus arteriosus in one, and major aorto-pulmonary collateral artery in one. We believe this technique can enhance the understanding of the causes of airway involvement and facilitate the creation of an appropriate surgical plan.