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A case of successful six consecutive deliveries in a 41-year-old woman with Uhl's anomaly.

Uhl's anomaly, or parchment right ventricle is a myocardial disorder of unknown cause that mainly involves the right ventricle. Uhl's anomaly may represent a cause of right heart dilatation, failure, and premature sudden death due to ventricular arrhythmias. Although most of the cases of Uhl's anomaly end fatally in infancy or childhood, a limited number of cases have been reported in advanced ages. Also, in pregnant women, this situation increases the risk to both mother and baby and requires special management. This is the first report of six successful consecutive gestations and vaginal deliveries without special managements in a patient with Uhl's anomaly.

Angiotensin-Converting Enzyme Inhibitors↗

Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment.

OBJECTIVE: To clarify the clinical features of systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH) and to evaluate the efficacy of immunosuppressive treatment. METHODS: Case records were reviewed for 194 patients with SLE who were admitted to Aoyama Hospital of Tokyo Women's Medical University between 1992 and 1999. There were 12 patients with PH [8 SLE and 4 SLE + systemic sclerosis (SSc) overlap syndrome]. These patients were compared with 59 age and sex matched patients with SLE for clinical characteristics and laboratory findings. The efficacy of treatments for PH was also evaluated. RESULTS: In our cohort of 194 patients with SLE, 6.2% had PH. The plasma thrombin-antithrombin III complex and plasma D-dimer levels were significantly higher in patients with PH compared with those without PH. Eight patients with PH (4 SLE and 4 SLE + SSc) were treated with corticosteroids (CS) +/- cyclophosphamide (CYC). Right ventricular systolic pressure (RVSP) was improved in 7 of 8 patients. In 6 of 7 responders to the therapy, the treatment was started as soon as they were diagnosed with PH. PH relapsed in 2 patients treated with oral CS +/- CS pulse therapy, but their RVSP was decreased again by immunosuppressive treatment. CONCLUSION: CS +/- CYC was effective for PH associated with SLE. Immunosuppressive treatment should be performed during the early stage of PH to improve prognosis.

Adult↗

Implantable left ventricular assist devices.

The first bridge to transplant with a left ventricular assist device (LVAD) was preformed over 20 years ago. Since that time, the devices have continued to evolve and now patients are being supported with devices as an alternative to transplantation. The primary indication of end-stage heart failure remains the same but increased knowledge about patient selection, the timing of implant, and patient management have contributed to improved outcomes with decreasing adverse events. Multiorgan failure, right ventricular failure, bleeding, infection, thromboembolism, and device malfunction continue to be the most serious threats to long-term survival in these patients. Despite that, patients who do well are now able to be discharged from the hospital and resume relatively normal lives with the devices. The article reviews 3 of the most widely used LVADs for bridge to transplant therapy: the Thoratec; HeartMate vented electric; and the Novacor Left Ventricular Assist System. Indications, mechanism of operation, clinical problems, and out-of-hospital preparation are outlined.

Cost-Benefit Analysis↗

Successful perioperative utilisation of inhaled nitric oxide in mitral valve surgery.

Pulmonary hypertension associated with mitral valve disease can significantly complicate mitral valve replacement. Nitric oxide (NO) has an established role in congenital heart surgery and in cardiac transplantation. However the evidence to date suggests that iNO does not provide a clinical advantage in pulmonary hypertension associated with valvular heart disease. We discuss a 56-year-old male with acute mitral incompetence who underwent emergency surgery. Following mitral valve replacement he was weaned off cardiopulmonary bypass with an intra-aortic balloon pump and high doses of inotropic support but continued to be hypotensive. Inspection of the heart showed right-sided dysfunction and evidence of pulmonary hypertension. Following the introduction of iNO he rapidly improved. He was successfully separated from the cardiopulmonary bypass support and subsequently made a full recovery and was discharged. We believe that this case demonstrates the utility of iNO in the management of pulmonary hypertension secondary to valvular disease in the acute setting. This is in contrast to the evidence against its utility in established pulmonary hypertension in chronic valvular heart disease. Further confirmation of this observation may lead to the therapeutic use of NO in this subpopulation of cardiac surgery patients.

Administration, Inhalation↗

Balloon dilation of right ventricular outflow tract in a dog with tetralogy of Fallot.

Balloon dilation was performed on a dog with tetralogy of Fallot. Immediately following balloon dilation, the peak systolic pressure gradient across the pulmonic valve declined from 97 to 63 mmHg. Doppler echocardiography following balloon dilation revealed increased pulmonary blood flow. Clinical symptoms obviously improved and the dog's improved condition was maintained for 4 months. There were no serious complications in performing the procedure. It was concluded that balloon dilation was a safe and effective treatment for a dog case with tetralogy of Fallot. Long-term follow-up studies will be required to identify the exact indications of balloon dilation for tetralogy of Fallot.

Animals↗

[Massive pulmonary capillary occlusion by microthrombi. Unexpected cause of fatal right heart failure during liver transplantation].

We report the case of a 46-year old man who developed an unexpected fatal cardiac failure during liver transplantation. Attempts at resuscitation were unsuccessful. At necropsy the lungs showed numerous microthrombi occluding small lung vessels and pulmonary capillaries. Thrombi were not found in other organs. The source of this extensive thrombus formation is not known. The thrombi could have been developed within the liver, the venous blood stream between liver and lungs or the pulmonary capillaries. In our experience, this complication is very rare, and a risk profile is not known.

Capillaries↗

[Double outlet right ventricle with sub-aortic obstruction caused by hypertrophy of the parietal band. Echographic image apropos of a case].

The authors report the case of an 18 month old infant with double-outlet right ventricle and subaortic ventricular septal defect with severe subaortic obstruction. Echocardiography showed the subaortic obstruction to be due to severe hypertrophy of the parietal band which grew upwards to the aortic annulus. A peak instantaneous Doppler gradient of 72 mmHg was recorded between the right ventricle and the aorta. The hypertrophy of the band was secondary to pulmonary artery banding performed in the neonatal period at the same time as cure of an isthmic coarctation of the aorta. Surgical exploration confirmed the echocardiographic findings a circular subaortic muscular stenosis related to hypertrophy of the parietal band. Resection of this band liberated the left ventricular outflow tract and made possible a tunnelisation procedure between the left ventricle and the aorta. Postoperative Doppler echocardiography showed the absence of subaortic stenosis.

Aortic Coarctation↗

Arrhythmogenic right ventricular dysplasia: MR features.

Arrhythmogenic right ventricular dysplasia (ARVD) is a heart disease characterized by a total or partial fat replacement of the myocardium. A total of 30 patients were studied with a suspected diagnosis of ARVD. Clinical criteria used for evaluation of ARVD were: (a) ventricular origin arrhythmias with a left bundle branch block configuration, (b) T-wave inversion in the anterior precordial leads, (c) ventricular kinetic alterations observed using echocardiography and angiography and (d) cardiac failure when there are no pathologies attributable to other heart diseases. All patients had serial EKG and echocardiography tests. One third of patients underwent angiocardiography; 7 of 30 had Holter; 7 of 30 had exercise test just to evaluate the effectiveness of the anti-arrhythmic therapy. All patients underwent MRI examination. The following MRI criteria were used: (a) high-intensity areas indicating the fatty substitution of the myocardium, (b) ectasia of the right ventricular outflow tract, (c) dyskinetic bulges, (d) dilation of the right ventricle and (e) enlargement of the right atrium. The diagnosis of ARVD was classified as highly probable for patients manifesting at least three positive criteria, probable with two positive criteria, dubious with one and negative in the absence of all criteria. Highly probable diagnosis of ARVD was made in 8 patients, probable in 4, dubious in 7 and negative in 11. The MRI technique is very effective in the assessment of ARVD. The MRI criteria may be helpful in the diagnosis of this condition.

Adipose Tissue↗

[Mobile thrombi of the right heart in pulmonary embolism].

Systematic transthoracic echocardiography in all cases of pulmonary embolism may demonstrate right heart thrombi. The results of this monocentric series of 28 consecutive cases observed between 1987 and 1996 were analysed. Twenty-four patients were in NYHA Class IV: thirteen were in cardiogenic shock. Echocardiographic signs of acute cor pulmonale were usually observed: 96.3% of patients had right ventricular dilatation, 85.2% paradoxical interventricular septal motion, 88.9% pulmonary hypertension. The thrombus was typical serpentine (27/28 cases) arising from the lower limb veins. Passage into the left heart chambers through a patent foramen ovale was observed in 3 cases. Pulmonary embolism was confirmed in all cases. This is an extreme therapeutic emergency and 13 patients (46.4%) died despite treatment: surgery (7/16), thrombolysis (2/5), heparin (3/4) or interventional radiology (1/3). After the acute phase, the prognosis was generally good, as demonstrated by the 100% survival rate at 28.6 +/- 25 months. This study confirms the gravity of mobile right heart thrombi in pulmonary embolism. The diagnosis is echocardiographic. No significant difference in mortality was observed between the different therapeutic approaches used in this series. The echocardiographic finding of these thrombi is a traditional indication for emergency surgical embolectomy. Thrombolysis is rapid and readily available and seems to provide promising results alone or before surgery. In patients with contraindications to thrombolysis, interventional radiology or simple heparin therapy may be proposed.

Adult↗

Safety of antiarrhythmics during pregnancy: case report and review of the literature.

A young woman is reported with intractable sustained ventricular tachycardia thought to originate in the right ventricle, which was treated successfully with encainide after failure to respond to beta-blockers and several class IA antiarrhythmic agents. She became pregnant twice while on encainide and gave birth to two healthy children. This is the first report of pregnancy during treatment with encainide. A literature review showed no other reported case of encainide taken during pregnancy, but several reports of the safe use of flecainide, a similar class IC drug, during pregnancy. Other antiarrhythmics are also reviewed.

Adolescent↗

[A new method of pulmonary artery cannulation with minimally invasive decannulation for right ventricular assistance:an animal experiment].

OBJECTIVE: To create a new method of pulmonary artery cannulation during operation for right ventricular assistance, for which minimally invasive decannulation can be done after operation. METHODS: A specially-made disassemblable composite graft consisting of a woven dacron tube and a bovine pericardial tube (composite graft) was anastomosed to the pulmonary artery as a pathway in seven dogs by median sternotomy. The end of the woven dacron tube and a link-thread for connecting the two tubes in the composite graft were drawn outside the body through an intercostal tunnel. A predisposed drawing-tieing thread in the composite graft was drawn outside through another intercostal tunnel and then tightened. An inflow cannula was placed into the left femoral vein or jugular vein via an incision of skin so as to establish a right ventricular assist system. By means of the centrifugal pump, the right ventricular bypass was established. After stopping the pumping, the 2 sets of predisposed prolene thread in the composite graft were tied by catheter and knotting tool via the small skin incision, 8 knots for each set, to close the bovine pericardial tube, and then the drawing-tieing thread, link-thread, and the dacron tube in the composite graft were removed. Median sternotomy was performed again to observe the anastomotic stoma, the closure of the stump of bovine pericardial tube, and whether hemorrhage occurred. RESULTS: The mean maximum output of right ventricular assist system from left femoral or jugular vein to pulmonary artery was (64.4 +/- 12.3) ml.min(-1).kg(-1). The second sternotomy showed satisfactory knotting on the bovine pericardial tube except in one case, whose second set of drawing-tieing thread was tied in the seventh knot and failed to be drawn out, thus partially remained in the body. No bleeding was found in all cases. CONCLUSION: The new cannulating methods with minimally invasive decannulation after operation created in this study is simple, easy and effective.

Animals↗

Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation.

The cases of five patients with previous Senning (n = 4) or Mustard (n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m2, versus 55.6 gm/m2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount (n = 1) or disappeared completely (n = 3).

Adult↗

Usefulness of multidetector row computed tomography to quantify right ventricular size and function in adults with either tetralogy of Fallot or transposition of the great arteries.

An ever-increasing number of patients with congenital heart disease are reaching adulthood; follow-up often requires right ventricular (RV) quantification. Complex anatomy, acoustic window, and active implants such as pacemakers may limit modalities, such as echocardiography and cardiac magnetic resonance imaging (MRI), in this population. Recent advances in multidetector row cardiac computed tomography (MDCT) allow multiphase, multislice reconstructions in any plane but have not been applied to RV quantification. Fourteen patients with tetralogy of Fallot or transposition of the great arteries underwent cardiac MRI and MDCT examinations, with comparable quantification of RV volumes and ejection fractions and no systematic error by Bland-Altman analysis using MRI as the gold standard.

Adolescent↗

Management of the failing systemic right ventricle.

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump. In cases where the left ventricle has prolonged exposure to low pressures in the pulmonary circulation, the left ventricle must be "retrained" to assume a systemic pressure load. Anatomic repair, with or without a preparatory period of left ventricular retraining, is a consideration for three clinically relevant scenarios: (1) patients with transposition of the great arteries after an atrial level switch (Senning or Mustard procedure), (2) patients with congenitally corrected transposition who are unoperated or who have undergone physiologic ("classic") repair, and (3) unoperated patients with transposition who present after the neonatal period.

Cardiac Surgical Procedures↗

Experience with right ventricular assist devices for perioperative right-sided circulatory failure.

BACKGROUND: Right-sided circulatory failure remains a significant source of morbidity and mortality for both cardiac transplant and left ventricular assist device recipients. METHODS: We reviewed our experience with 11 patients who required a right ventricular assist device (RVAD) after either orthotopic heart transplantation or left ventricular assist device implantation. Variables analyzed included total time of RVAD support, hemodynamic and hematologic parameters, and parameters of end-organ perfusion. These were assessed at five time points: (1) at least 2 weeks before RVAD implantation, (2) intraoperatively just before RVAD insertion, (3) while on RVAD support, and, for those who survived, (4) just before RVAD explantation, and (5) off RVAD support. Survival was assessed as the ability to be weaned successfully from RVAD support. Urine output and serum transaminase levels were recorded throughout the period of RVAD support. RESULTS: Five patients received an ABIOMED 5000 BVS RVAD, and 6 received a Bio-Medicus centrifugal pump. Nine patients in the study underwent orthotopic heart transplantation and had development of right-sided circulatory failure from 0 to 96 hours after donor organ insertion, and 2 patients underwent left ventricular assist device implantation 12 to 48 hours before RVAD support. The mean time of RVAD support for survivors was 133.6 +/- 33.6 hours (range, 107 to 190 hours). Six patients were successfully separated from RVAD support, and 5 patients died while on RVAD support. Causes of death included sepsis (2), biventricular failure (2), and coagulopathy (1). Continuous arteriovenous hemodialysis was employed in 3 of 6 survivors and 1 of 5 nonsurvivors. CONCLUSIONS: Right ventricular assist devices work most effectively if implanted early enough to avoid significant, potentially irreversible end-organ injury. We liberally employ continuous arteriovenous hemodialysis, minimize the use of heparin immediately postoperatively, keep patients sedated, and continue RVAD support until the patient displays signs of hemodynamic and end-organ recovery as heralded by (1) a decrease in central venous pressure and, more importantly, a decrease in pulmonary artery diastolic pressure, (2) an increase in urine output, and (3) a decrease in serum transaminase levels.

Adolescent↗

Preoperative risk factors for right ventricular failure after implantable left ventricular assist device insertion.

BACKGROUND: Implantable left ventricular assist device (LVAD) insertion complicated by early right ventricular (RV) failure has a poor prognosis and is generally unpredictable. METHODS: To determine preoperative risk factors for perioperative RV failure after LVAD insertion, patient characteristics and preoperative hemodynamics were analyzed in 100 patients with the HeartMate LVAD (Thermo Cardiosystems, Inc, Woburn, MA) at the Cleveland Clinic. RESULTS: RV assist device support was required for 11 patients (RVAD group). RVAD use was significantly higher in younger patients, female patients, smaller patients, and myocarditis patients. There was no significant difference in the cardiac index, RV ejection fraction, or right atrial pressure between the two groups preoperatively. The preoperative mean pulmonary arterial pressure (PAP) and RV stroke work index (RV SWI) were significantly lower in the RVAD group (p = 0.015 and p = 0.011, respectively). Survival to transplant was poor in the RVAD group (27%) and was 83% in the no-RVAD group. CONCLUSIONS: The need for perioperative RVAD support was low, only 11%. Preoperative low PAP and low RV SWI were significant risk factors for RVAD use.

Adult↗

Pulmonary artery distensibility in pulmonary arterial hypertension: an MRI pilot study.

Pulmonary arterial hypertension (PAH) is a disease of the small vessels in which there is a substantial increase in pulmonary vascular resistance leading to right ventricle failure and death. Invasive haemodynamic evaluation is mandatory not only for diagnosis confirmation but also to address prognosis and eligibility for the use of calcium-channel blockers through an acute vasodilator challenge. Noninvasive surrogate response markers to the acute vasodilator test have been sought. In the present study, the relationship between pulmonary artery distensibility, assessed using magnetic resonance imaging (MRI), and response to acute vasodilator tests was investigated. In total, 19 patients diagnosed with idiopathic PAH without any specific treatment were evaluated. Within a 48-h window after pulmonary artery catheterisation, patients underwent cardiac MRI. Cardiac index, calculated after the determination (invasively and noninvasively) of cardiac output, showed excellent correlation, as did right atrial pressure and right ventricle ejection fraction. Pulmonary artery distensibility was significantly higher in responders. A receiver operating characteristic curve analysis has shown that 10% distensibility was able to differentiate responders from nonresponders with 100% sensitivity and 56% specificity. The present findings suggest that magnetic resonance imaging and pulmonary artery distensibility may be useful noninvasive tools for the evaluation of patients with pulmonary hypertension.

Administration, Inhalation↗

Greater impairment of right ventricular systolic function in patients with anterior myocardial infarction because of the extent of proximal lesions.

The present study examined the influence of the extent of the ischemic area on right ventricular (RV) systolic function and the relation between the RV global and regional systolic function in patients with anteroseptal myocardial infarction (MI). Biplane right ventriculography was performed in 15 subjects as the control group, and 46 patients with anteroseptal MI as the MI group. Three dimensions of the RV (the long axis dimension [LA], the anterior-posterior dimension [AP] and the septum-free wall dimension [SF]) were examined to assess regional function The MI group had a larger right ventricular end-systolic volume index and lower right ventricular ejection fraction than the control group. The more proximal the coronary lesion, the lower was the ejection fraction of the RV in the MI group. The MI group had lower percent shortening (% shortening) of the SF than the control group, but there were no significant change in the % shortening of AP and LA between the groups. The results suggest that the degree of impairment of RV systolic function depends on the extent of the infarcted area, and that the impairment is mainly from a reduction in the %shortening of the SF.

Aged↗

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