Platypnoea-orthodeoxia syndrome secondary to patent foramen ovale (PFO): a challenging subset for PFO percutaneous closure.

BACKGROUND: Platypnoea-Orthodeoxia Syndrome (P-OS) is a rare disease characterised by arterial desaturation exacerbated by the upright position and relieved by recumbency. Patent foramen ovale (PFO) may lead to a P-OS causing a right-to-left shunt in the course of particular diseases that induce atrial deformation. Percutaneous closure of the defect usually allows prompt improvement of the clinical status. METHODS: A series of patients with P-OS was treated with percutaneous PFO closure, according to standard clinical practice. Procedural monitoring was performed by transoesophageal two-dimensional-echocardiography (2D-echo). RESULTS: PFO percutaneous closure was initially effective in only half of the patients because of high rates of acute residual shunt. This unexpected result was related to the very complex anatomy evaluation with 2D-echo, mainly due to a peculiar atrial deformation occurring in P-OS. A second device delivery allowed it to achieve complete defect closure in the remaining patients. Technical issues arising during the procedures are widely discussed. CONCLUSIONS: Percutaneous closure of PFO in patients with P-OS is feasible but some technical issues should be considered when PFO anatomy is not clear with traditional imaging techniques.

Cardiovascular Magnetic Resonance from Fetal to Adult Life-Indications and Challenges: A State-of-the-Art Review.

Cardiovascular magnetic resonance (CMR) imaging offers a comprehensive, non-invasive, and radiation-free imaging modality, which provides a highly accurate and reproducible assessment of cardiac morphology and functions across a wide spectrum of cardiac conditions spanning from fetal to adult life. It minimises risks to the patient, particularly the risks associated with exposure to ionising radiation and the risk of complications from more invasive haemodynamic assessments. CMR utilises high spatial resolution and provides a detailed assessment of intracardiac and extracardiac anatomy, ventricular and valvular function, and flow haemodynamic and tissue characterisation, which aid in the diagnosis, and, hence, with the management of patients with cardiac disease. This article aims to discuss the role of CMR and the indications for its use throughout the different stages of life, from fetal to adult life.

Cardiac Imaging in Patients After Fontan Palliation: Which Test and When?

The Fontan operation represents the final stage of a series of palliative surgical procedures for children born with complex congenital heart disease, where a "usual" biventricular physiology cannot be restored. The palliation results in the direct connection of the systemic venous returns to the pulmonary arterial circulation without an interposed ventricle. In this unique physiology, systemic venous hypertension and intrathoracic pressures changes due to respiratory mechanics play the main role for propelling blood through the pulmonary vasculature. Although the Fontan operation has dramatically improved survival in patients with a single ventricle congenital heart disease, significant morbidity is still a concern. Patients with Fontan physiology are in fact suffering from a multitude of complications mainly due to the increased systemic venous pressure. Consequently, these patients need close clinical and imaging monitoring, where cardiac exams play a key role. In this article, we review the main cardiac imaging modalities available, summarizing their main strengths and limitations in this peculiar setting. The main purpose is to provide a practical approach for all clinicians involved in the care of these patients, even for those less experienced in cardiac imaging.

Echocardiographic two-dimensional speckle tracking identifies acute regional myocardial edema and sub-acute fibrosis in pediatric focal myocarditis with normal ejection fraction: comparison with cardiac magnetic resonance.

The aim here was to describe the role of speckle tracking echocardiography (STE), in identifying impairment in systolic function in children and adolescents with focal myocarditis and without reduction in ejection fraction. We describe data from 33 pediatric patients (age 4-17 years) admitted for focal myocarditis, confirmed by cardiac magnetic resonance (CMR), and without impaired ejection fraction and/or wall motion abnormalities. All children underwent Doppler echocardiography examination with analysis of global (G) and segmental longitudinal strain (LS) and CMR for the quantification of edema and myocardial fibrosis. Reduction in LS was defined according to age-specific partition values. At baseline, impaired GLS was present in 58% of patients (n = 19), albeit normal ejection fraction. LS was also regionally impaired, according to the area of higher edema at CMR (i.e. most impaired at the level of the infero-lateral segments as compared to other segments (p < 0.05). GLS impairment was also moderately correlated with the percentage edema at CMR (r = - 0.712; p = 0.01). At follow-up, GLS improved in all patients (p < 0.001), and normal values were found in 13/19 patients with baseline reduction. Accordingly persistent global and regional impairment was still observed in 6 patients. Patients with persistent LS reduction demonstrated residual focal cardiac fibrosis at follow-up CMR. Both global and regional LS is able to identify abnormalities in systolic longitudinal mechanics in children and adolescents with focal myocarditis and normal ejection fraction. The reduction in LS is consistent with edema amount and localization at CMR. Furthermore, LS identifies regional recovery or persistent cardiac function impairment, possibly related to residual focal fibrosis.

Laparoscopic liver resection for hepatocellular carcinoma in Fontan-associated chronic liver disease. The first case report.

INTRODUCTION: A well-recognized long-term complication after Fontan procedure (FP), a complex cardiac surgery performed in patients with univentricular hearts, is the development of chronic liver disease and hepatocellular carcinoma (HCC). Due to the risk of cardiac and liver decompensation, liver resection of HCC is challenging and the laparoscopic approach has never been reported. PRESENTATION OF THE CASE: We present the first case of laparoscopic liver resection (LLR) of HCC in a 33-years-old girl with cardiac-related cirrhosis after FP. Intraoperatively, the pneumoperitoneum was established at 8-10 mmHg and adequate fluid infusion was given to maintain the cardiac preload. After an ultrasound-guided thermoablation along the free-tumor margin of the hepatic lesion, a full laparoscopic non-anatomical resection of the tumor in segment V was performed, without Pringle manouver and blood transfusion requirement. The cardiac function remained stable during the surgery and thereafter, and the post-operative course was uneventful. DISCUSSION: HCC in chronic liver disease after FP is associated with high-risk mortality. Due to the complex hemodynamic changes after FP, open surgical resections often aren't feasible and loco-regional percutaneous treatment or combined liver-heart transplantation are the only therapeutic options. This case suggests that LLR in FP patients has low-risk of liver and cardiac decompensation, minimizing the pneumoperitoneum insufflation to ensure low intra-abdominal/intra-thoracic pressures and providing accurate anaesthetic management to maintain proper cardiac preload and output. CONCLUSION: LLR for HCC after FP is safe and feasible, and might be considered an alternative treatment of HCC for which the best treatment has not been defined yet.

Closure of patent foramen ovale: when and how?

Percutaneous closure of a patent foramen ovale (PFO) was performed in 98 consecutive patients (mean age 52.5 +/- 13 years, 61 women). Indications included recurrent transient ischaemic attack (47%), cryptogenic stroke (34%), peripheral embolism (11%), disabling migraine with aura (4%), professional scuba diving (1 pt) and severe platypnea-orthodeoxia syndrome (1 pt). Each PFO was characterized by transesophageal echocardiography (TEE) according to anatomy, degree of shunt (1-mild, 2-moderate, 3-severe), right atrial anatomical features relevant for PFO closure (such as presence of an Eustachian valve, Chiari network, lipomatosis or absence of septum secundum) with a new classification scheme. According to this classification successful device delivery was obtained in 100% of pts. Major complications included heparin-induced thrombocytopenia in 1 pt and device dislodgment in 1 pt; minor complications were mostly related to the catheter introduction site (2 pts) and mild immediate shunt (2 pts). In conclusion, percutaneous PFO closure based on strict anatomic criteria is a safe procedure with minimal periprocedural complications.

Drug-eluting stents for the percutaneous treatment of the anastomosis of the left internal mammary graft to left anterior descending artery.

BACKGROUND: Data on the treatment of left internal mammary to left anterior descending artery (LIMA-to-LAD) anastomotic disease are scarce and not homogeneous. Both surgery and percutaneous interventions (PCI) have been attempted, but the most effective treatment has not yet been established. In particular, should PCI be performed, the role of stenting seems to be limited by less favorable results than in other subsets of lesions. OBJECTIVE: To assess the clinical impact of drug-eluting stent (DES) use in this particular subset of lesions. METHODS: We describe a cohort of patients treated with PCI on LIMA-to-LAD anastomoses, reporting acute 1-year clinical and angiographic outcomes. The clinical impact of DES use was evaluated as the requirement for target lesion revascularizations (TLR). RESULTS: Fifty-six consecutive patients were evaluated. Acute procedural success was achieved in 52 patients (92.8%). Plain balloon angioplasty allowed acute procedural success in 15 patients (28.8%), whereas stenting was required in 37 patients (71.2%) with suboptimal results or to treat complications. Bare-metal stents (BMS) were used in 17 and DES in 20 patients, without differences in acute results. One-year clinical follow-up was available in 96.1% of patients. TLR were needed in 17.3% of patients. No significant differences were detected in TLR rates after treatment with BMS and DES (26.6% vs. 25%; P=0.99). Two late stent thromboses were observed after DES deployment. CONCLUSION: PCI of the stenoses of LIMA-to-LAD anastomoses with DES did not provide any clinical improvement over BMS use in long-term outcomes; DES use was associated with some cases of late thrombosis.

Inflammatory substrate with eosinophils may be present in bare-metal stent thrombosis.

Coronary stent thrombosis is a rare but life-threatening event after percutaneous revascularization. We report an in-vivo documentation of the pathologic process in the setting of a very late thrombosis within a bare-metal stent, showing eosinophil infiltrates. Interestingly, our case indicates that eosinophil infiltrates might not be a peculiarity of late drug-eluting stent thrombosis.

"Gender paradox" in outcome after percutaneous coronary intervention with paclitaxel eluting stents.

AIMS: To evaluate in-hospital and long-term outcome of women undergoing percutaneous coronary intervention (PCI) with paclitaxel-eluting stents (PES) as compared to men and historic controls treated with bare metal stents. METHODS AND RESULTS: Eight-hundred and ten (810) consecutive patients (642 men and 168 women) with PES were analysed and also compared with 3,515 patients (2,811 men and 704 women) with at least one bare metal stent in the three years previous. In the PES group, women were significantly older than men (mean age of 68+/-10 vs. 63+/-10 years, p<0.001), with more diabetes (39.9 vs. 29.7%, p<0.05), smaller treated vessels (<2.5 mm in 34.9 vs. 24.8%, p=0.003), more left-anterior descending lesions (56.6 vs. 43.2%, p<0.001). More men had multivessel disease (78.8 vs. 62.5%, p<0,001), dyslipidaemia (73.2 versus 65.4%, p<0.05), smoking habit (74.3 versus 34.5%, p<0.001) or prior revascularisation (49.7 versus 36.1%, p<0.05). Compared to bare metal stents, similar higher risk features were recorded in women over time. No gender specific differences in in-hospital outcome were observed. At 12 months the rates of cardiac death, acute myocardial infarction (AMI) and late thrombosis were respectively 2.0%, 3.7% and 0% in women and 2.1%, 3.2%, and 1.2% in men (all p=NS). CONCLUSIONS: Albeit with worse baseline clinical and angiographic characteristics in women, very high immediate success rates and a positive outcome can be achieved in both sexes after PES.

Immediate removal of femoral-sheath following protamine administration in patients undergoing intracoronary paclitaxel-eluting-stent implantation.

BACKGROUND: Immediate sheath-removal using post-procedural reversal of heparin with protamine reduces groin complications, shortens bed rest and hospital stay after percutaneous coronary intervention (PCI) with bare-metal stents. No data are available with newer and possibly more thrombogenic paclitaxel-eluting stents (PES). AIM: We assessed the safety and efficacy of post-procedural protamine administration after successful coronary PES implantation in elective PCI and in patients with acute coronary syndromes (ACS). METHODS: A consecutive series of 291 patients received 0.5 mg of protamine per 100 units of heparin whenever the post-procedural ACT was > 180 seconds, followed by immediate removal of the sheath (protamine group). Outcomes were compared to a historic control group comprising 291 consecutive patients, who also underwent PCI with PES, but without reversal of anticoagulation by protamine (non protamine group). The incidence of post-procedural vascular complications and bleeding complications, as well as hospital stay, were compared; as were the incidence of major cardiac events at 24 h, 30 days and 6 months. RESULTS: The post-procedural bleeding complications were significantly higher in the non-protamine group. Vascular complications were also more frequent in patients who were not treated with protamine. Hospitalisation length was significantly lower in the protamine group than in the non-protamine group (13.6 +/- 7 h versus 20.41 +/- 3.9 h; p < 0.001). The protamine-group patients also had a significantly reduced bed rest (10.3 h +/- 5.6 h versus 18 h +/- 3.5 h; p < 0.001). During hospitalisation, after PES implantation, no deaths or acute stent thrombosis were observed in either group. The overall incidence of thrombosis and major adverse cardiac events at follow-up were similar in the two groups. CONCLUSIONS: Immediate heparin neutralisation by protamine after successful PES implantation appears to be safe and feasible, also in patients with ACS. Use of protamine and early sheath removal after PCI confers early deambulation and may significantly limit healthcare cost, reduce vascular complications, bedrest, delayed discharge and patient discomfort.