We report a case of posterior non-arteritic ischaemic optic neuropathy (NAION) causing bilateral visual loss in a 7-month-old female infant, after a therapeutic course with sildenafil, a phosphodiesterase type 5 inhibitors (PDE5i). The patient was affected by a complex cyanotic congenital heart defect and had undergone cavopulmonary anastomosis (Glenn operation) 3 months ago. After 2 months of recurring chylothorax, a course of oral sildenafil was administered, with the hypothesis that pulmonary vascular resistances were increased. Approximately 4 weeks later the acute onset of visual worsening and poor pupillary light reflex prompted the diagnosis of posterior NAION. Despite a rapid cessation of PDE5i and systemic treatment with corticosteroids, no visual recovery was noticed at 2-year follow-up. NAION has been associated with PDE5i therapy in adults, but to the best of our knowledge it is almost unheard of in children. We suggest close monitoring of visual function in children undergoing treatment with sildenafil.
A 7-year-old (y.o.) boy was referred to our center for a specialist evaluation due to alteration of the ventricular repolarization in the anterior and lateral lead on the electrocardiogram at medical evaluation for physical activity. The child was totally asymptomatic, with normal growth and unremarkable past medical history. The physical examination revealed only S4 and a minimal apical systolic murmur. Transthoracic echocardiogram demonstrated a mildly dilated left ventricle (LV) and normal left ventricular ejection fraction. Strain pattern of basal-medium segments of lateral wall was reduced. Right coronary artery (RCA) appeared dilatated and the left coronary artery (LCA) showed an inverse flow at Doppler evaluation. Findings were suspicious for ALCAPA diagnosis (Anomalous Left Coronary Artery Arising from the Pulmonary Artery); a rare congenital heart disease that, if not treated, leads to death in 90% of cases during first year of life. Adult type of ALCAPA (adolescence-60 y.o) is even more rare and can cause heart failure due to a dilatative cardiomyopathy, chronic cardiac ischemia and, in 80-90% of cases, sudden cardiac death. We performed a coronary angiography that confirmed the anomalous origin of LCA from the pulmonary artery. The RCA was dominant, enlarged and supplied an important collateral flow for LCA. Coronary angiography is indicated not only for diagnosis confirmation, but also for coronary tree demonstration, especially in adult type of ALCAPA, because of the presence of not rare anomalies or “adaptation” of peripheral coronary circulation. All these features are important positive prognostic factors that enable survival beyond infancy. LCA ostium stenosis or bronchial artery collateral vessels are also common but weren't seen in this case. In adult type of ALCAPA surgery is usually indicated at the time of diagnosis, so a coronary button transfer and pulmonary Valsalva sinus reconstruction with autologous pericardial patch were performed. This is the preferred surgical technique in adult patients as it is the most anatomic correction. Since before hospitalization heart failure therapy with betablocker was introduced and after surgery aspirin therapy was started too. Post-surgery transthoracic echocardiogram highlighted a moderate pericardial effusion without haemodynamic impact and a pleural effusion, so an anti-inflammatory therapy with ibuprofen, cortisone and then colchicine was started. After discharge anti-inflammatory therapy was under-titrated until suspension based on gradual reduction of pericardial and pleural effusion. Patient was always free of symptoms and with good performance status. ALCAPA or Bland–White–Garland syndrome is an uncommon congenital heart disease. The presence of adequate collateral circulation between RCA and LCA allows to survive beyond infancy (adult type). We described a case of early diagnosis of adult type of ALCAPA in asymptomatic child thanks to a careful examination of basal electrocardiogram for evaluation of non-competitive sport practice.
Background Common Truncus arteriosus (TA) is a rare congenital, cyanotic heart disease with a single vessel coming out from both ventricles and related low interventricular defect, right-left shunt, low pulmonary flow and right ventricle (RV) hypertension. TA is associated with aortic coarctation (CoA) in 10-20% of cases. Case A 15-year-old male affected by type 2 TA and CoA undergoing first post-natal cardiac surgery with pulmonary valved conduit (PVC) implantation and CoA repair, subsequently multiple percutaneous (PC) interventions due to right pulmonary artery (RPA) stenosis and re-CoA. He was referred to our Centre for dyspnoea on mild efforts. Echocardiography revealed RV hypertension (75% of systemic pressure) and mild disfunction. Angio-CT showed RPA re-stenosis due to peeling of previous stents, a degenerative PVC and mild residual CoA. Results Cardiac catheterization confirmed imaging findings, revealing the following systolic/diastolic blood pressures [mmHg]: 100/15 in RV, 33/10 in left pulmonary artery (LPA), 13/7 in RPA, 132/65 in ascending and 125/60 in descending aorta. First, extensive balloon-interrogation of stented RPA and RV outflow tract (RVOT) with semi-compliant balloon was performed followed by PC intra-stenting transluminal angioplasty of RPA with modified undersized non-compliant balloon. As RPA was very closed to CoA with risk of aortic rupture, and mostly the main pulmonary OT accounted for increased pressure gradient, RPA enlargement with re-stenting was avoided. Then, a long semi-opened-cell stent was arranged on the prosthetic RVOT and Melody valve was implanted as Valve-in-Valve (ViV), both with balloon post-dilatation. Dilated LPA with only moderate focal stenosis and mild residual CoA were excluded from intervention. Post-interventional right heart pressures were reduced: 45/4 in RVOT and 42/13 mmHg in the main PA. At 1 month dyspnoea disappeared with good effort tolerance, echocardiographic ViV mean gradient was 12 mmHg with improved RV systolic function and pressure (50% of systemic). Conclusions Repaired complex TA of type described may develop multiple re-stenosis of RPA. RPA optimization was not the goal of the procedure because at high risk of vessels’ injury due to the very closed anatomy with otherwise prosthetic CoA. RV pressures and dysfunction could depend on degenerative obstructive PVC. Thus, interventional approach mostly focused on the main PA aimed at working PVC could be the best effective treatment.
Background: Percutaneous valve implantation (PPVI) is now considered feasible and safe especially in patients with conduit between the right ventricle and the pulmonary arteries. However there are still no clear indications on valvolization in native outflow tract (RVOT), traditionally considered at risk because of the dynamic characteristics of the RVOT Aim: The aim of this study is to demonstrate the feasibility and safety of building a percutaneous conduit using a stent in the native RVOT before valve implant performing PPVI two months after stent implant, on the basis of previous animal studies, where endotelitation of the stent was shown to be reached within 60 days. Methods: From October 2010 to April 2014, 19 pts, with tetralogy of Fallot, (mean age 15 ± 6 yrs) previously corrected by transannular patch technique, underwent a cardiac catheterization in order to build a stent-conduit in the native RVOT. After a complete right catheterization a balloon was inserted through a long sheath in the RVOT. During the inflation of the balloon a right ventricular angiography and a left coronary artery angiography are performed. In the presence of a complete occlusion of the RVOT and no coronary compression, a stent was deployed in the RVOT. Two months later an Edwards Sapien Pulmonary valve was implanted. Results: Twenty two stents were implanted in 19 pts. The mean time of the procedures was 185 ± 89 min with a mean fluoroscopy time of 72 ± 33 min. Two main complications occurred: in 1 pt the stent partially occluded the right pulmonary artery and in a second pt the stent was dislocated in RVOT. In both cases the patients were treated surgically. In all other patients the procedure was successful and patients were discharged home two days later, on Aspirin therapy. The correct position of the stent was confirmed at the 1 month of follow-up by echocardiographic examination. Among the 19 pts , 152 underwent implantation of Edwards Sapien Pulmonary Valve, two months later. No complications occurred during the procedure and during the FU. Two patients are still waiting PPVI procedure Conclusions: A two-step procedure approach increases the safety of PPVI with no impact on clinical conditions or complications
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