Levosimendan In Single Ventricle Heart Failure After Longterm Survival Of A Modified Blalock-Taussig Shunt.

We report the case of a 44 year-old patient with complex ACHD, admitted with acute decompensated heart failure (ADHF) in hemodynamic profile B. He had a single ventricle with pulmonary atresia, previously submitted to three modified Blalock-Taussig shunts (mBTs) at the age of 2, 12 and 19 years old. Despite conventional treatment with diuretics, ß-blockers (BB) and isosorbide dinitrate the patient progressed to profile C and the transthoracic echocardiogram disclosed a reduced systolic function. Likewise, levosimendan was commenced and an appropriate decongestion and a marked reduction in the NT-proBNP were seen. Treatment with angiotensin-converting-enzyme inhibitor, BB, ivabradine and mineralocorticoid receptor was optimized. The patient was discharged home after 26 days in NYHA class III and referred for heart transplant after right heart catheterization. To our knowledge, this is the first report of successful levosimendan's use in ADHF in a mBTs long-term survivor.

Advantages of a prospective multidisciplinary approach in transcatheter aortic valve implantation: Eight years of experience.

INTRODUCTION: Aortic stenosis is the most prevalent type of valvular disease in Europe. Surgical aortic valve replacement (SAVR) is the standard therapy, while transcatheter aortic valve implantation (TAVI) is an alternative in patients at unacceptably high surgical risk. Assessment by a heart team is recommended by the guidelines but there is little published evidence on this subject. The purpose of this paper is to describe the experience of a multidisciplinary TAVI program that began in 2008. METHODS: The heart team prospectively assessed 473 patients using a standardized approach. A total of 214 patients were selected for TAVI and 80 for SAVR. Demographic, clinical and procedural characteristics and long-term success rates were compared between the groups. RESULTS: TAVI patients were older than the SAVR group (median 83 vs. 81 years), and had higher surgical risk scores (median EuroSCORE II 5.3 vs. 3.6% and Society of Thoracic Surgeons score 5.1 vs. 3.1%), as did the patients under medical treatment only. These scores were unable to assess multiple comorbidities. Patients' outcomes were different between the three groups (mortality with SAVR 25% vs. TAVI 37.6% vs. conservative therapy 57.6%, p=0.001). CONCLUSIONS: The heart team program was able to select candidates appropriately for TAVI, SAVR and conservative treatment, taking into account the risk of both invasive treatments. The use of a prospective standardized heart team approach is recommended, but requires continuous monitoring to ensure effectiveness in a timely manner.

Mechanical Thrombectomy for Stroke After Cardiac Surgery.

Stroke after cardiac surgery remains a devastating complication and its treatment options are limited. Systemic fibrinolysis is a relative contraindication, because it raises the risk of systemic hemorrhage. Endovascular therapy, mechanical thrombectomy, and intra-arterial fibrinolysis have emerged as safer options. We present three patients who developed strokes following cardiac surgery who underwent successful mechanical thrombectomy and review the literature on this subject. doi: 10.1111/jocs.12776 (J Card Surg 2016;31:517-520).

[Transapical aortic valve replacement: a 5-years experience]. / VÁLVULA AÓRTICA PERCUTÂNEA TRANSAPICAL: 5 ANOS DE EXPERIÊNCIA.

OBJECTIVES: Transcatheter aortic valve replacement (TAVR) by transapical approach is accepted for severe aortic stenosis in patients with high risk for conventional surgical therapy. Herein is reported the initial clinical results of this technique in a reference center METHODS: We conducted a longitudinal prospective single center study including 54 consecutive patients (mean age 79 ± 7.5 years, 59% male) who underwent, between November 2008 and October 2013, TAVR with Edwards Sapien valves throught transapical approach. The etiology was native aortic stenosis in 83% (mean gradient = 49 ± 18.3 mmHg and area = 0,7 cm2), 11% aortic disease and 3 patients had degenerated biological valvular prostheses, being 65% in class III/IV NYHA. The major comorbidities were coronary heart disease in 56% (status post-coronary surgery 37%), diabetes (37%), peripheral artery disease (31%) and chronic renal failure (24%). The logistic EuroSCORE was 19.8 ± 11.2 and EuroSCORE II 5.5 ± 3.5%, with STS mortality score 5.1 ± 3.7 and 23.7 ± 7.6 STS morbidity. RESULTS: Analysis following the VARC-2 criteria (Valve Academic Research Consortium), showed 30-day mortality of 5.6%; peri-procedural myocardial infarction 7.4%; disabling cerebral vascular accident 1.9%; severe haemorrhage 14.8%; major vascular complications 5.6%; pacemaker implantation 11%. Unplanned extracorporeal circulation was used in 5 cases and prosthetic dysfunction occurred in 4 patients (shift valve in 2 cases). The median hospital stay was 8.0 days, with re-hospitalization in 12.2% of cases. The composite VARC-2 endpoints were: device success = 90.7%; early safety at 30 days = 75.9% and clinical efficacy after 30 days= 83.7%. CONCLUSION: The transapical approach was found as an effective therapy for patients at high risk for conventional surgery.

[Total anomalous pulmonary venous connection: a 17-years experience of a single pediatric cardiology center]. / Retorno venoso pulmonar anómalo total: 17 anos de experiência de um centro de cardiologia pediátrica.

OBJECTIVE: We reviewed our patients who underwent surgical repair for total anomalous pulmonary venous connection at our hospital. MATERIAL AND METHODS: All patients with total anomalous pulmonary venous connection who underwent correction from February 1995 to October 2012 were included in this retrospective analysis. Clinical data was collected on presentation,morphology, associated cardiac lesions, echocardiography and cardiac cathetherization results, surgical repair and postoperative course. RESULTS: There were 25 patients in the cohort, median age 19 days. The type of total anomalous venous connection was supracardiac in fifteen, cardiac in five, infracardiac in four, mixed-type in one patient. Six patients had single-ventricle anatomy with atrial isomerism in five. All patients with biventricular anatomy had cardiac defects associated: interauricular communication(17), patent arterial duct (9), tricuspid insufficiency (3), pulmonary stenosis (2), interventricular communication (1), mitral valve atresia (1), aortic arch hypoplasia (1). Nine patients (36%) presented with pulmonary venous obstruction. Respiratory distress was the most frequent symptom on presentation (80%), five patients were invasively ventilated. One patient was treated with extracorporeal membrane oxygenation prior to surgical correction. Twenty four cases were diagnosed with transthoracic echocardiography alone. Nine patients were evaluated with angiography that confirmed the echocardiographic diagnosis in eight patients; it allowed diagnosis of total anomalous pulmonary venous connection in one patient. Twenty four patients were submitted to surgery in the first six months of live (68% in neonatal period), one patient was operated at 27 years. Total anomalous pulmonary venous connection was corrected at first surgery in 22 patients. Two patients died after surgical correction,one with infracardiac total anomalous pulmonary venous connection with obstruction and another with supracardiac type with complex cardiac anomaly. There were no deaths in the last eleven years (14 patients). Postrepair pulmonary venous obstruction was diagnosed in one patient who was reoperated successfully. CONCLUSION: Echocardiography provides adequate diagnostic data on total anomalous pulmonary venous connection so that surgery can be performed without angiography in most cases. Early surgical correction of total anomalous pulmonary venous connection has acceptable mortality and good long-term postrepair outcome.

Multidetector CT of rare isolated partial anomalous pulmonary venous return.

A 16-year-old male was referred to our clinic for evaluation of pulmonary hypertension. An isolated anomalous pulmonary venous return of both left pulmonary veins to the innominate vein was diagnosed after echocardiographic and multidetector CT (MDCT) evaluation. Three-dimensional MDCT image reconstructions were used to better clarify this rare type of disease and adequate choice of surgical approach.

[DiGeorge syndrome and vascular ring. An unusual association with multidisciplinary approach]. / Síndrome de DiGeorge e anal vascular. Associação invulgar e abordagem multidisciplinar.

Velo-cardio-facial syndrome/DiGeorge/CATCH 22 is a spectrum of association, characterized by unusual face, cleft or incompetent palate, congenital heart disease with defects of the outflow tracts, absence of the thymus and parathyroid glands, often associated with developmental and behavioral disorders. This association is caused by a microdeletion in chromosome band 22q11.2. In a 4-month-old infant, with obstructive lower respiratory distress and poor weight gain since 2 months of age, truncus arteriosus was diagnosed and surgically corrected. On the postoperative period maintained dependency on mechanical ventilation, with persistent hypoventilation of the left lung. Fiberoptic bronchoscopy revealed complete obstruction of the left main bronchus by an extrinsic compression due to a vascular ring diagnosed by cardiac catheterization that showed a common anomalous origin of both right and left subclavian arteries and the ligamentum arteriosum. A second surgery by left lateral thoracotomy corrected the vascular ring. The maintenance of the collapse of the left main bronchus led to selective endobronchial stenting. The migration of the stent to the trachea, with acute respiratory distress, required emergent endoscopic removal of the stent. Thereafter, the evolution was uneventful. The association of DiGeorge syndrome with vascular ring is unusual. Unexpected evolution in these patients require a multidisciplinary technical approach for diagnosis and eventual emergent intervention.

Atrial tachycardia ablation in a patient with double outlet right ventricle corrected by surgery.

The development of surgical and percutaneous techniques for treatment or palliation of congenital heart disease has prolonged survival in these patients and has increased late complications, particularly arrhythmias. Such arrhythmias are more frequently refractory to medical therapy, requiring percutaneous ablation. We present the clinical case of a 14-year-old child with complex congenital heart disease (double outlet right ventricle) who underwent two corrective surgeries (Rastelli operation and subsequent replacement of the homograft in the conduit connecting the right ventricle to the pulmonary artery; ventricular septal defect closure and tricuspid valve repair). After the second surgery the patient presented with wide complex syncopal tachycardia, refractory to medical therapy. Electrophysiologic study (EPS) identified an isthmus-dependent atrial flutter that was successfully treated by radiofrequency (RF) ablation (a linear block was created along the cavo-tricuspid isthmus). Three months later a new episode of tachycardia occurred, but without syncope. The second EPS revealed an atrial tachycardia originating from the lateral wall of the right atrium, which was treated by ablation with focal application of RF energy. Four months after the last EPS the child remains free of arrhythmic symptoms, under no anti-arrhythmic therapy.

Nitric oxide in the management of pulmonary hypertension in the context of cardiac surgery in the adult.

Pulmonary hypertension is a significant problem to take into account in the post-operative management of cardiac patients, especially valvular patients. Inhaled nitric oxide allows more effective control of pulmonary pressure and other hemodynamic parameters, with better post-operative results. We present a clinical case of a patient with mitral stenosis and severe pulmonary hypertension, with post-operative hemodynamic instability, in which we used inhaled nitric oxide for better control of pulmonary pressures and to help ventilator weaning.

Atrial fibrillation and effective refractory period of the pulmonary vein ostia.

BACKGROUND: The pathogenesis of atrial fibrillation (AF) is not completely understood. The role of pulmonary veins (PV) in AF initiation is documented, and the recent demonstration of persistent fibrillatory activity in an isolated PV suggests that the PV play a role in the maintenance of AF. OBJECTIVE: Since AF is facilitated by multiple reentry circuits in atrial tissue with short effective refractory periods (ERP) and prolonged conduction times, we investigated whether PV have shorter ERP compared with the left atrium (LA). POPULATION AND METHODS: The study population consisted of five male patients, between 45 and 78 years of age, with normal sized LA; three had coronary artery bypass surgery (and no previous history of atrial arrhythmias) and two had paroxysmal lone AF refractory to antiarrhythmic drugs and were referred for percutaneous ablation with radiofrequency energy. In the surgical patients, epicardial bipolar pacing wires were inserted in the PV ostia and LA roof at the end of the procedure. Post-operatively, the pacing wires were used to determine atrial thresholds and ERP in the PV ostia and LA roof. In the AF patients, atrial thresholds and ERP at these locations were obtained with the mapping/ablation catheter before and after PV isolation. ERP were determined with a basic pacing cycle length of 500 ms and a single extrastimulus with an initial coupling interval of 350 ms, gradually decreased (10 ms at a time) until atrial capture failure or AF induction. RESULTS: ERP in the LA roof were longer than 210 ms. The shortest ERP was always obtained in a PV ostium, with the shortest in the left PV ostia. The ERP values of the right inferior PV most resembled those of the LA roof. In patients referred for ablation, AF was induced when PV ostia with ERP shorter than 200 ms were stimulated. CONCLUSION: In the present study, the ERP of PV ostia were shorter than LA ERP, possibly explaining not only the presence of ectopic foci in the PV ostia, but also sustained fibrillatory activity in isolated PV, despite conversion of the atria to sinus rhythm. This fact may also explain the higher success rate and the preference for PV isolation in AF ablation.