Can aura migraine be elicited by isolated pulmonary arteriovenous fistula?-A case report.

A pulmonary arteriovenous fistula (PAVM) is an abnormal blood vessel that creates a direct connection between a pulmonary artery and its tributary vein bypassing capillary filter, establishing as a consequence of a low-resistance right-to-left shunting (RLS). The vast majority of PAVMs are congenital appearing more often in females than in males. A great number of patients with PAVMs is suffering concurrently from hereditary hemorrhagic telangiectasia (HHT) whose incidence is around 1 in 5,000. Very few cases of acquired PAVMs have been described in the literature. Paradoxical embolism through PAVMs can cause systemic desaturation, cyanosis, and serious cerebrovascular ischemic events (transient ischemic attacks, strokes, and intracranial abscess), even when the abnormal blood vessel is small (diameter <3 mm). Notably, it has been reported a high prevalence of aura migraine (MHA) symptoms in patients with PAVMs and concomitant HHT. We described in this study the case of a young aura migraineur female patient without HHT in whom isolated PAVM below the detection limit of pulmonary angiography and chest computed tomography angiography (CTA) has been documented by contrast Transthoracic and Transesophageal Echocardiography (cTTE/TEE) showing a delayed (>17 s) RLS coming from left pulmonary veins to left atrium while a patent foramen ovale (PFO), small atrial septal defects or septum primum fenestration could not be detected despite several attempts. Contrast Transcranial Doppler (cTCD) confirmed a delayed (>16 s) RLS with two short "shower" patterns corroborating the diagnosis of an extra-cardiac RLS. During the right heart catheterization and pulmonary angiography, it was impossible to cross the interatrial septum with a multipurpose catheter. The patient was finally discharged with off-label thienopyridine agents (clopidogrel 75 mg die) in terms of primary prophylaxis for paradoxical right-to-left embolization of thrombotic material ultimately. Aura migraine symptoms were nearly abolished by P2Y12 platelet inhibition, suggesting a platelet-based mechanism. During 2 years of clinical follow-up on thienopyridine therapy, the resolution of aura migraine episodes was definitively accomplished with significant improvement in her quality of life.

Outsourcing the Remote Management of Cardiac Implantable Electronic Devices: Medical Care Quality Improvement Project.

BACKGROUND: Remote management is partially replacing routine follow-up in patients implanted with cardiac implantable electronic devices (CIEDs). Although it reduces clinical staff time compared with standard in-office follow-up, a new definition of roles and responsibilities may be needed to review remote transmissions in an effective, efficient, and timely manner. Whether remote triage may be outsourced to an external remote monitoring center (ERMC) is still unclear. OBJECTIVE: The aim of this health care quality improvement project was to evaluate the feasibility of outsourcing remote triage to an ERMC to improve patient care and health care resource utilization. METHODS: Patients (N=153) with implanted CIEDs were followed up for 8 months. An ERMC composed of nurses and physicians reviewed remote transmissions daily following a specific remote monitoring (RM) protocol. A 6-month benchmarking phase where patients' transmissions were managed directly by hospital staff was evaluated as a term of comparison. RESULTS: A total of 654 transmissions were recorded in the RM system and managed by the ERMC team within 2 working days, showing a significant time reduction compared with standard RM management (100% vs 11%, respectively, within 2 days; P<.001). A total of 84.3% (551/654) of the transmissions did not include a prioritized event and did not require escalation to the hospital clinician. High priority was assigned to 2.3% (15/654) of transmissions, which were communicated to the hospital team by email within 1 working day. Nonurgent device status events occurred in 88 cases and were communicated to the hospital within 2 working days. Of these, 11% (10/88) were followed by a hospitalization. CONCLUSIONS: The outsourcing of RM management to an ERMC safely provides efficacy and efficiency gains in patients' care compared with a standard in-hospital management. Moreover, the externalization of RM management could be a key tool for saving dedicated staff and facility time with possible positive economic impact. TRIAL REGISTRATION: ClinicalTrials.gov NCT01007474; http://clinicaltrials.gov/ct2/show/NCT01007474.

Thalassemia and heart surgery: aortic valve repair after endocarditis.

Outcome after heart valve surgery in patients affected by thalassemia is an unreported issue and to the best of our knowledge only 7 cases have been described in the literature. Heart valve disease is commonly encountered in thalassemia patients and heart valve replacement carries high risk of prosthesis complications including thrombosis and embolization despite optimal anticoagulation management. We report a successful long-term outcome after a case of aortic valve repair after mycotic valve endocarditis.

[Isolated non-compaction of left ventricle in childhood: clinical experience with 5 cases]. / Non compattazione isolata del ventricolo sinistro in età pediatrica: esperienza clinica su cinque casi.

BACKGROUND: Isolated noncompaction of the left ventricular myocardium, a rare congenital disorder, is thought to be due to an arrest of myocardial morphogenesis. It is characterized by an excessively prominent trabecular meshwork and by deep intertrabecular recesses. The aim of this study was to clarify the late outcome of this malformation, reporting our experience on 5 pediatric patients observed for a period of 11 years. METHODS: We describe the clinical, electrocardiographic and echocardiographic findings of 5 pediatric cases, with an age ranging from 1 month to 10 years. The mean follow-up was 4.5 years (> 10 years in 1 patient). RESULTS: In no case did life-threatening ventricular arrhythmias or systemic embolic events occur. Four patients who developed heart failure have been submitted to digoxin, diuretic, angiotensin-converting enzyme and anticoagulant therapy. One of them is still asymptomatic and in good health. One patient died while on the waiting list for cardiac transplantation. CONCLUSIONS: The appropriate and early diagnosis and treatment of this disorder can improve the outcome even though further studies will be needed to elucidate its long-term prognosis. At the same time, in view of the high incidence of a positive family history, evaluation of all members of the family is warranted.

Apical ventricular septal defect with reverse bidirectional transseptal flow: a sign of restriction within the right ventricle.

Apical ventricular septal defects (VSD) may spontaneously become restricted or may even close following tissue outgrowth within the right ventricle in the region of the apical muscle bundles. We report a case of spontaneous restriction of an apical VSD localized within the right ventricle in the region of prominent apical trabeculae, in which pulsed Doppler interrogation showed an unusual pattern of bidirectional blood flow across the VSD, with right to left flow in systole and left to right in diastole. Angiography confirmed these unusual aspects. This reverse bidirectional transseptal flow is due to the physiological incorporation of the apical part of the right ventricular cavity in the left ventricle and may be, in the absence of right outflow obstruction, a sign of restriction of the VSD inside the right ventricle.