Validation of intracardiac shunt using thoracic bioimpedance and inert gas rebreathing in adults before and after percutaneous closure of atrial septal defect in a cardiology research unit: study protocol.

INTRODUCTION: Intrathoracic shunt quantification is a major factor for appropriate clinical management of heart and pulmonary diseases. Intracardiac shunts quantified by pulmonary to systemic output ratio (Qp/Qs) are generally assessed by Doppler echocardiography, MRI or catheterisation. Recently, some authors have suggested the concomitant use of thoracic bioimpedance (TB) and inert gas rebreathing (IGR) techniques for shunt quantification. The purpose of this study is to validate the use of this approach under conditions where shunt fraction is directly quantified such as in patients with isolated atrial septal defect (ASD). METHODS AND ANALYSIS: This trial is a prospective, observational single-centre, non-blinded study of adults seen for percutaneous closure of ASD. Qp/Qs ratio will be directly measured by Doppler echocardiography and direct Fick. IGR and TB will be used simultaneously to measure the cardiac output before and after closure: the ratio of outputs measured by IGR and TB reflecting the shunt fraction. The primary outcome will be the comparison of shunt values measured by TB-IGR and Doppler echocardiography. ETHICS AND DISSEMINATION: The study has been approved by an independent Research Ethics Committee (2017-A03149-44 Fr) and registered as an official clinical trial. The results will be published in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: NCT03437148; Pre-results.

Validation of cadmium-zinc-telluride camera for measurement of left ventricular systolic performance.

BACKGROUND: There are paucity of data comparing measurements of left ventricular systolic performance using cadmium-zinc-telluride (CZT) semiconductor cameras with other imaging modalities. This study compared the new system with echocardiography (echo) and cardiac magnetic resonance (CMR) imaging. METHODS: 60 Patients presenting with ST-elevated myocardial infarction (MI) were included. Each patient underwent echo, myocardial perfusion imaging using Spectrum Dynamics D-SPECT(r) (CZT-SPECT), and CMR 6 weeks after MI. The primary endpoint was the agreement between CZT-SPECT and CMR for left ventricular ejection fraction (LVEF) measurement. RESULTS: 48 of the 60 patients underwent all 3 studies (echo, CMR, and CZT-SPECT) 40 days after admission. CZT-SPECT and CMR LVEF were well correlated (r = .79, P < .0001), as well as CZT-SPECT vs echo and CMR vs echo (r = .79 and .84, respectively, P < .0001). The segmental LV wall thickening and wall motion also showed good concordance between three techniques. CONCLUSIONS: CZT-SPECT is reliable for LVEF measurement.

Amiodarone-Induced Thyrotoxicosis Recurrence After Amiodarone Reintroduction.

Reintroduction of amiodarone in patients with a history of amiodarone-induced thyrotoxicosis (AIT) is rarely used. To date, the risk of AIT recurrence after amiodarone reintroduction is unpredicted. The aim of the study was to evaluate the risk of AIT recurrence. Retrospectively, from 2000 to 2011, all euthyroid patients with a history of AIT with amiodarone reintroduction were included. Type and severity of the first AIT, amiodarone chronology, and thyroid function evolution after reintroduction of amiodarone were investigated: 46 of 172 patients with AIT history needed amiodarone reintroduction. At first AIT episode, the mean age was 62.2 ± 16 years with male gender predominance; 65% of patients were classified as type 1 AIT. AIT recurred in 14 patients (30%), 12 patients developed hypothyroidism (26%), and 20 patients remained euthyroid (44%). Characteristics of type 1 AIT during the first episode, namely briefer exposure period to amiodarone and longer duration of treatment to normalize thyroid hormones, were predictive of AIT recurrence; 73% of patients (8 of 11) with previous episode of type 1 AIT, who did not receive a preventive thioamide treatment, developed a second episode of AIT. Thioamide preventive treatment could be useful to prevent type 1 AIT recurrence. In conclusion, AIT recurrence after amiodarone reintroduction is 4 times more frequent in patients with type 1 AIT history. Thyroid ablation before amiodarone reintroduction in patients with a history of type 1 AIT is preferred. Preventive thioamide treatment could be suggested in patients with type 1 AIT history pending for surgery.

Acute and Chronic Pheochromocytoma-Induced Cardiomyopathies: Different Prognoses?: A Systematic Analytical Review.

Pheochromocytoma and paraganglioma (PPG) are rare and late-diagnosed catecholamine secreting tumors, which may be associated with unrecognized and/or severe cardiomyopathies. We performed a computer-assisted systematic search of the electronic Medline databases using the MESH terms "myocarditis," "myocardial infarction," "Takotsubo," "stress cardiomyopathy," "cardiogenic shock", or "dilated cardiomyopathy," and "pheochromocytoma" or "paraganglioma" from 1961 to August 2012. All detailed case reports of cardiomyopathy due to a PPG, without coronary stenosis, and revealed by acute symptoms were included and analyzed. A total of 145 cases reports were collected (49 Takotsubo Cardiomyopathies [TTC] and 96 other Catecholamine Cardiomyopathies [CC]). At initial presentation, prevalence of high blood pressure (87.7%), chest pain (49.0%), headaches (47.6%), palpitations (46.9%), sweating (39.3%), and shock (51.0%) were comparable between CC and TTC. Acute pulmonary edema (58.3% vs 38.8%, P = 0.03) was more frequent in CC. There was no difference in proportion of patients with severe left ventricular systolic dysfunction (LV Ejection Fraction [LVEF] < 30%) at initial presentation between both groups (P = 0.15). LVEF recovery before (64.9% vs 40.8%, P = 0.005) and after surgical resection (97.7% vs 73.3%, P = 0.001) was higher in the TTC group. Death occurred in 11 cases (7.6%). In multivariate analysis, only TTC was associated with a better LV recovery (0.15 [0.03-0.67], P = 0.03). Pheochromocytoma and paraganglioma can lead to different cardiomyopathies with the same brutal and life-threatening initial clinical presentation but with a different recovery rate. Diagnosis of unexplained dilated cardiomyopathy or TTC should lead clinicians to a specific search for PPG.

Effectiveness of anticoagulant therapy in the treatment of post-TAVI bioprosthetic thrombosis.

Bioprosthetic thromboses are rarely reported in post-transcatheter aortic valve implantation (TAVI). We describe herein the case of bioprosthetic valve thrombosis in an 82-year-old patient which resolved completely after anticoagulant therapy.

Kawasaki shock syndrome complicating a recurrence of Kawasaki disease.

We describe a case of recurrent Kawasaki disease (KD) in a non-Asian 6-year-old boy who had been diagnosed with typical KD without cardiac involvement at age 3 years. He was admitted to the PICU 3 years later for heart failure, hypotension, and deterioration of his general condition. Ultrasonography revealed left ventricular dysfunction with a 44% ejection fraction and grade I mitral valve failure without coronary artery involvement. Subsequent observation of hyperemic conjunctiva, bilateral cervical adenopathies with erythematous skin (normal neck ultrasound and computed axial tomography findings), peeling of the fingertips at day 8 of the illness, and occurrence of an inflammatory syndrome led to a diagnosis of incomplete recurrent KD with a clinical picture of Kawasaki shock syndrome (KSS). Clinical improvement was rapidly obtained after intravenous immunoglobulin and intravenous corticosteroid therapy (30 mg/kg per day for 3 subsequent days). Left ventricular function gradually improved, with ultrasound returning to normal after 3 months. Diagnosis was difficult to establish because of the recurrence of the disease and the incomplete clinical picture, with clinical features of KSS. Physicians need to be aware of these pitfalls in the management of patients with clinical signs of KD.

A single pathophysiological pathway in Takotsubo cardiomyopathy: Catecholaminergic stress.

BACKGROUND: Takotsubo cardiomyopathy (TTC) continues to be under-diagnosed, due to its varying presentation, with potentially serious consequences if treatment is delayed. AIMS: To demonstrate the consistent involvement of catecholaminergic stress in TTC, regardless of the trigger. METHODS: Between 01 July 2009 and 31 August 2013, patients managed in our centre for thoracic pain syndrome, with or without troponin release, were followed up prospectively. TTC was diagnosed from the apical ballooning seen on left ventricular imaging (angiography or transthoracic echocardiography) in the absence of a significant coronary artery lesion. Triggers (emotional trauma, surgical stress and ß2-mimetic intoxication) were recorded; catecholamine-secreting tumours were screened for with a urinary methoxylate-derivative assay. RESULTS: TTC was diagnosed in 40 out of 2754 (1.5%) patients with thoracic pain syndrome, with or without troponin release. Triggers were emotional trauma (n=29, 72.5%), surgical stress (n=5, 12.5%), adrenergic intoxication (n=3, 7.5%) and catecholaminergic tumour (n=3, 7.5%). Mean left ventricular ejection fraction at admission was 38.0 ± 15.7%. Eight (20%) patients initially showed cardiogenic shock. In-hospital mortality was 7.5%, with no deaths from cardiogenic causes. Thirty-five (94.6%) of the survivors had recovered a normal left ventricular ejection fraction (> 55%) by discharge. CONCLUSION: Whatever the trigger, the common denominator in TTC is catecholaminergic stress. Classically suggested after emotional trauma, TTC may also be induced by surgical stress or endogenous or iatrogenic ß2-mimetic intoxication. The various contexts all have a similarly excellent cardiovascular prognosis if treated early.

Mitochondrial protein synthesis is increased in oxidative skeletal muscles of rats with cardiac cachexia.

Since cardiac cachexia could be associated with alterations in muscular mitochondrial metabolism, we hypothesized that the expected alterations in the activities of mitochondrial oxidative enzymes could be associated with changes in mitochondrial protein synthesis in oxidative skeletal muscles. Cardiac cachexia was provoked in male rats by the ligation of the left coronary artery. Six cachectic and 6 control rats were age-paired, and their food intake was observed. The synthesis of mitochondrial proteins was measured by [1-13C]-valine infusion in soleus, tibilais, myocardium, and liver. Muscles (soleus, gastrocnemius, and tibialis anterior), heart, kidneys, liver, and visceral adipose tissue were weighed. Mitochondrial cytochrome c oxydase IV as well as citrate synthase and myosin ATPase activities were measured. As expected, decreased food intake was observed in the cachectic group. Heart, kidney, and liver weights were higher in the cachectic group, while the visceral adipose tissue weight was lower (P < .01). No changes in muscle weights were observed. Soleus mitochondrial proteins fractional synthesis rate was higher in the cachectic group (P = .054). Cytochrome c oxydase IV activity was reduced (P = .009) and increased (P = .038) in the soleus and liver of the cachectic rats, respectively. No change in citrate synthase activity was observed. Myosin ATPase activity was reduced in the gastrocnemius of the cachectic group (P < .01). Mitochondrial protein synthesis is increased in the soleus of rats with cardiac cachexia, suggesting a compensatory mechanism of the impaired oxidative mitochondrial function. Further work should assess whether the mitochondrial protein synthesis is altered in chronic heart failure patients with cardiac cachexia, and whether this is the cause or the consequence of cachexia.

Should an implanted defibrillator be considered in patients with vasospastic angina?

UNLABELLED: Vasospastic angina is a frequent and well-recognized pathology with a high risk of life-threatening ventricular arrhythmias and sudden cardiac death. The diagnosis of vasospastic angina requires the combination of clinical and electrocardiographic variables and the results of provocation tests, such as ergonovine administration. Smoking cessation is the first step in the management of vasospastic angina. Optimal medical treatment using calcium-channel blockers and/or nitrate derivatives can provide protection, but life-threatening ventricular arrhythmias may occur despite optimal medical treatment and several years after the start of treatment. In this review, we evaluate the role of implantable defibrillators as a complement to optimal medical management in patients with life-threatening ventricular arrhythmias due to vasospastic angina; this role is not well characterized in the literature or guidelines. We discuss the role of implantable defibrillators in secondary prevention in light of three recent cases managed in our departments and a review of the literature. An implantable defibrillator was implanted in two of the three cases of vasospastic angina with ventricular arrhythmias that we managed. We considered secondary prevention by implantable defibrillator to be justified even in the absence of any obvious risk factor. Ventricular arrhythmias recurred during implantable defibrillator follow-up in the two patients implanted. CONCLUSION: In patients with life-threatening ventricular arrhythmias due to vasospastic angina, an implantable defibrillator should be considered because of the risk of recurrence despite optimal medical management.

Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block.

BACKGROUND: The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. METHODS AND RESULTS: A multicenter retrospective study (13 French referral centers, from 1980-2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0 ± 6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively (P<0.001). A long PR interval was found in 18.5% of the parents but never in control subjects (P<0.0001). Complete or incomplete right bundle-branch block was observed in 39.2% of the parents and 1.5% of the control subjects (P<0.0001). Complete or incomplete left bundle-branch block was found in 15.4% of the parents and 3.1% of the control subjects (P<0.0006). Estimated heritability for isolated conduction disturbances was 91% (95% confidence interval, 80%-100%). SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. CONCLUSIONS: ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Alcohol septal ablation through a bare metal stent after rotative atherectomy: a complex procedure.

We report the case of a patient with severe malignant hypertrophic obstructive cardiomyopathy (HOCM) and calcified stenosis of the proximal and middle left anterior descending (LAD) coronary artery. We elected to treat his ischemic heart disease first. We performed angioplasty of the proximal and middle LAD, after rotative atherectomy, and implanted two bare metal stents. Thirty days later we treated his HOCM by alcohol septal ablation with catheterization of the first septal branch through the mesh of the bare metal stent implanted in the LAD. To our knowledge, this is the first documented report of such a procedure.

Early improvement in peripheral vascular tone following smoking cessation using nicotine replacement therapy: aortic wave reflection analysis.

OBJECTIVES: Cigarette smoking induces cardiovascular pathology and worsens arterial stiffness. Arterial stiffness can be assessed noninvasively based on 2 indices: pulse-wave velocity (PWV), indicating aortic stiffness, and the augmentation index (AIx), indicating aortic wave reflection. The impact of smoking cessation (SC) with nicotine replacement therapies (NRT) on arterial stiffness remains unknown. METHODS: AIx and PWV were studied prospectively (using a SphygmoCor system) in 26 long-term smokers (>10 cigarettes/day; mean age 43 ± 6 years) before (visit 1) and 28 and 56 days (visit 2 and visit 3) after SC supported by NRT. Abstinence was ascertained when exhaled carbon monoxide (CO) was <10 ppm; the quantity of NRT absorbed was assessed based on the serum cotinine level. RESULTS: Sixteen of 24 patients (67%) were abstinent at the end of the study; 8/24 had cut down on smoking, and 2 were lost to follow-up. CO and serum cotinine levels fell after SC. The mean AIx for the population as a whole was 23.4% at visit 1, with a significant early reduction by visit 2 (16.2%) and visit 3 (13.9%) (ANOVA, p < 0.001). The PWV, peripheral blood pressure, and heart rate were unchanged. CONCLUSIONS: The improvement in AIx and the stability of PWV after SC with NRT indicate improved peripheral vascular tone. This impact may account for the early clinical benefit of SC observed even when associated with NRT.

Kawasaki disease in adults: report of 10 cases.

Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). At the time of clinical presentation, complete KD was diagnosed in 4 patients, while 4 patients fulfilled the criteria for incomplete KD. For 3 of the 4 patients with incomplete KD, presence of severe inflammation, laboratory findings (hypoalbuminemia, anemia, elevation of alanine aminotransferase, thrombocytosis after 7 days, white blood cell count > or =15,000/mm, and urine > or =10 white blood cell/high power field), or echocardiogram findings were consistent with the diagnosis. In 2 patients, the diagnosis of KD was made retrospectively in the presence of myocardial infarction due to coronary aneurysms, after an undiagnosed medical history evocative of incomplete KD. Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.

Significant persistent ductus arteriosus in infants less or equal to 6 kg: percutaneous closure or surgery?

BACKGROUND: Percutaneous closure of large persistent ductus arteriosus using the Amplatzer duct occluder is an alternative to surgery. However, this device is not recommended in infants weighing less than 6 kg. AIM: To evaluate the safety and effectiveness of this procedure in low-body-weight infants. METHODS: We reviewed retrospectively data for infants weighing less or equal to 6 kg who underwent percutaneous closure of significant persistent ductus arteriosus using the Amplatzer duct occluder in France between 1998 and 2007. RESULTS: Data for 58 patients (mean weight: 5 kg, range: 3.4-6; mean age: 5.5 months, range: 2.1-15.3) were reviewed. Mean angiographic persistent ductus arteriosus minimal diameter was 3.7 mm (range: 1-7.5). Implantation of the Amplatzer duct occluder was successful in 89.7% of cases. In six (10.3%) patients, the device was not implanted because it would have led to significant aortic obstruction. One procedure-related death occurred in a 4 kg infant (1.7%). Major and minor complications occurred in 6.9 and 31.0% of patients, respectively. Persistent ductus arteriosus diameter greater than 3.7 mm, type C (tubular shape) and diameter/patient weight ratio greater than 0.91 were significantly associated with an unsuccessful procedure and/or major complications. During a median 10-month follow-up, no late device embolization occurred. CONCLUSIONS: Although percutaneous closure of significant persistent ductus arteriosus with the Amplatzer duct occluder is effective in low-body-weight infants, the level and severity of complications indicate surgery as first-line treatment, at least until further studies are done to assess the safety and effectiveness of the new Amplatzer duct occluder II in low-body-weight infants.

Asymptomatic congenital atresia of the left inferior pulmonary vein: a case report.

We present an unusual fortuitously discovered case of atresia of the left inferior pulmonary vein with drainage via anastomosis to the left upper pulmonary vein and the left atrium. Atresia of the pulmonary veins is a very rare anomaly that results from defective incorporation of the pulmonary venous system into the left atrium. The initial diagnosis, drawn from plain radiographs, was confirmed by cardiac catheterization and pulmonary angiography. The appearance of clinical signs, prognosis, and the need for treatment depend on how well substitute drainage is performed.

Simple repair of aortico-left ventricular tunnel in a newborn with early prenatal diagnosis.

We report a case with very early antenatal diagnosis of aortico-left ventricular tunnel (ALVT) at 22 weeks of pregnancy. Early recognition of ALVT by antenatal ultrasound allows prompt neonatal management and avoidance of harmful aortic valve regurgitation. The present case is unique because of the conjunction of very early antenatal diagnosis, prompt postnatal management, early surgical repair on the sixth day of life, direct closure from the aorta of the aortic orifice only, and optimal postoperative course with excellent mid-term result.

Efficiency of transcatheter closure of atrial septal defects in small and symptomatic children.

OBJECTIVES: We report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group. PATIENTS: We included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa. RESULTS: Transcatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down's syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain. CONCLUSION: Transcatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.

Right atrial tear associated with a tumour in the right atrium after blunt chest trauma.

Occult cardiac injury following blunt trauma is more common than generally suspected. Myocardial contusion is not rare, however, it is generally a benign disorder which often remains undiagnosed. We report a case of a right atrial rupture after blunt chest trauma causing a tamponade. A 24-year-old man was involved in a violent car accident and he presented in a state of collapse. A multislice computed tomography indicated a pericardial effusion (Figure 1). A transthoracic echocardiography was performed and confirmed pericardial effusion which was hyperechoic (Figure 2, Movie 1). Concerns about a possible mass in the right atrium led to examination with transesophageal echocardiography (Figure 3, Movie 2) which revealed the presence of a voluminous mass in the right atrium. The patient successfully underwent cardiac surgery to remove the mass, identified as a blood clot, and to repair the atrial tear. The present case is of special interest because of the rarity of documented incidents of blunt chest trauma causing right atrial tear and illustrates the usefulness of transesophageal echocardiography in completing the diagnosis in the event of haemopericardium.

Regressive aortic valve and infundibular tumors during idiopathic hypereosinophilic syndrome.

Fibroblastic endocarditis is a classic complication of prolonged hypereosinophilic syndrome, whatever the cause. In France, it is most frequently encountered in cases of idiopathic hypereosinophilic syndrome. It commonly involves the apex of the ventricles, with a clinical picture of restrictive cardiomyopathy of unfavorable prognosis, and the auriculoventricular valves. We report the case of a 77-year-old man in whom atypical cardiac involvement disclosed idiopathic hypereosinophilic syndrome. In addition to the usual features of obliteration of the apex and restrictive cardiomyopathy, echocardiographic examination showed severe left ventricular dysfunction and intracardiac tumors, one of which was unusually localized to an aortic valve. Treatment, which comprised strict control of the eosinophilic process, standard treatment for cardiac failure, and anticoagulation therapy, produced rapid and long-lasting improvement of his clinical status and left systolic and diastolic ventricular function, and on echocardiography the intracardiac tumors had totally disappeared. The patient suddenly died of septic shock 16 months after first being seen.