La cardiomiopatia aritmogena come causa di scompenso cardiaco e trapianto di cuore

Scopo dello studio: la cardiomiopatia aritmogena (CA) è conosciuta come causa di morte improvvisa, la sua relazione con lo scompenso cardiaco (SC) è stata scarsamente indagata. Scopo dello studio è la definizione della prevalenza e incidenza dello SC, nonché della fisiopatologia e delle basi morfologiche che conducono i pazienti con CA a SC e trapianto di cuore. Metodi: abbiamo analizzato retrospettivamente 64 pazienti con diagnosi di CA e confrontato i dati clinici e strumentali dei pazienti con e senza SC (NYHA III-IV). Abbiamo analizzato i cuori espiantati dei pazienti sottoposti a trapianto presso i centri di Bologna e Padova. Risultati: la prevalenza dello SC alla prima osservazione era del 14% e l’incidenza del 2,3% anno-persona. Sedici pazienti (23%) sono stati sottoposti a trapianto. I pazienti con SC erano più giovani all’esordio dei sintomi (46±16 versus 37±12 anni, p=0.04); il ventricolo destro (VD) era più dilatato e ipocinetico all’ecocardiogramma (RVOT 41±6 versus 37±7 mm, p=0.03; diametro telediastolico VD 38±11 versus 28±8 mm, p=0.0001; frazione di accorciamento 23%±7 versus 32%±11, p= 0.002). Il ventricolo sinistro (VS) era lievemente più dilatato (75±29 ml/m2 versus 60±19, p= 0.0017) e globalmente più ipocinetico (frazione di eiezione = 35%±14 versus 57%±12, p= 0.001). Il profilo emodinamico dei pazienti sottoposti a trapianto era caratterizzato da un basso indice cardiaco (1.8±0.2 l/min/m2) con pressione capillare e polmonare tendenzialmente normale (12±8 mmHg e 26±10 mmHg). L’analisi dettagliata dei 36 cuori dei pazienti trapiantati ha mostrato sostituzione fibro-adiposa transmurale nel VD e aree di fibrosi nel VS. Conclusioni: Nella CA lo SC può essere l’unico sintomo alla presentazione e condurre a trapianto un rilevante sottogruppo di pazienti. Chi sviluppa SC è più giovane, ha un interessamento del VD più severo accanto a un costante interessamento del VS, solo lievemente dilatato e ipocinetico, con sostituzione prevalentemente fibrosa.

Diagnosis of fatal pulmonary fat embolism with minimally invasive virtual autopsy and post-mortem biopsy

We report a case of a 78-year-old female with a proximal femur fracture caused by an accidental fall who died suddenly 1h after orthopaedic prosthesis insertion. Post-mortem computed tomography (CT) scan and histological examination of samples obtained with post-mortem percutaneous needle biopsies of both lungs were performed. Analysis of the medical history and the clinical scenario immediately before death, imaging data, and biopsy histology established the cause of death without proceeding to traditional autopsy. It was determined to be acute right ventricular failure caused by massive pulmonary fat embolism. Although further research in post-mortem imaging and post-mortem tissue sampling by needle biopsies is necessary, we conclude that the use of CT techniques and percutaneous biopsy, as additional tools, can offer a viable alternative to traditional autopsy in selected cases and may increase the number of minimally invasive forensic examinations performed in the future.

Pulse-pressure variation and hemodynamic response in patients with elevated pulmonary artery pressure: a clinical study

Pulse-pressure variation (PPV) due to increased right ventricular afterload and dysfunction may misleadingly suggest volume responsiveness. We aimed to assess prediction of volume responsiveness with PPV in patients with increased pulmonary artery pressure.

Pulse pressure variation and volume responsiveness during acutely increased pulmonary artery pressure: an experimental study

We found that pulse pressure variation (PPV) did not predict volume responsiveness in patients with increased pulmonary artery pressure. This study tests the hypothesis that PPV does not predict fluid responsiveness during an endotoxin-induced acute increase in pulmonary artery pressure and right ventricular loading.

[Myocardial dysfunction in sepsis]

Myocardial dysfunction appears in 25% of patients with severe sepsis and in 50% of patients with septic shock, even in the presence of hyper dynamic states. It is characterized by a reduction in left ventricle ejection fraction, that reverts at the seventh to tenth day of evolution. Right ventricular dysfunction and diastolic left ventricular dysfunction can also appear. There is no consensus if an increase in end diastolic volume is part of the syndrome. High troponin or brain natriuretic peptide levels are associated with myocardial dysfunction and a higher mortality. The pathogenesis of myocardial dysfunction is related to micro and macro circulatory changes, inflammatory response, oxidative stress, intracellular calcium management disturbances, metabolic changes, autonomic dysfunction, activation of apoptosis, mitochondrial abnormalities and a derangement in catecholaminergic stimulation. Since there is no specific treatment for myocardial dysfunction, its management requires an adequate multi systemic support to maintain perfusion pressures and systemic flows sufficient for the regional and global demands.

Atrial fibrillation in congestive heart failure

Atrial fibrillation (AF) and heart failure (HF) are common and interrelated conditions, each promoting the other, and both associated with increased mortality. HF leads to structural and electrical atrial remodeling, thus creating the basis for the development and perpetuation of AF; and AF may lead to hemodynamic deterioration and the development of tachycardia-mediated cardiomyopathy. Stroke prevention by antithrombotic therapy is crucial in patients with AF and HF. Of the 2 principal therapeutic strategies to treat AF, rate control and rhythm control, neither has been shown to be superior to the other in terms of survival, despite better survival in patients with sinus rhythm compared with those in AF. Antiarrhythmic drug toxicity and poor efficacy are concerns. Catheter ablation of AF can establish sinus rhythm without the risks of antiarrhythmic drug therapy, but has important procedural risks, and data from randomized trials showing a survival benefit of this treatment strategy are still lacking. In intractable cases, ablation of the atrioventricular junction and placement of a permanent pacemaker is a treatment alternative; and biventricular pacing may prevent or reduce the negative consequences of chronic right ventricular pacing.

Risk stratification of normotensive patients with acute symptomatic pulmonary embolism

Treatment guidelines recommend strong consideration of thrombolysis in patients with acute symptomatic pulmonary embolism (PE) that present with arterial hypotension or shock because of the high risk of death in this setting. For haemodynamically stable patients with PE, the categorization of risk for subgroups may assist with decision-making regarding PE therapy. Clinical models [e.g. Pulmonary Embolism Severity Index (PESI)] may accurately identify those at low risk of overall death in the first 3 months after the diagnosis of PE, and such patients might benefit from an abbreviated hospital stay or outpatient therapy. Though some evidence suggests that a subset of high-risk normotensive patients with PE may have a reasonable risk to benefit ratio for thrombolytic therapy, single markers of right ventricular dysfunction (e.g. echocardiography, spiral computed tomography, or brain natriuretic peptide testing) and myocardial injury (e.g. cardiac troponin T or I testing) have an insufficient positive predictive value for PE-specific mortality to drive decision-making toward such therapy. Recommendations for outpatient treatment or thrombolytic therapy for patients with PE necessitate further development of prognostic models and conduct of clinical trials that assess various treatment strategies.

Catheter-directed ultrasound-accelerated thrombolysis for the treatment of acute pulmonary embolism

Systemic thrombolysis rapidly improves right ventricular (RV) dysfunction in patients with acute pulmonary embolism (PE) but is associated with major bleeding complications in up to 20%. The efficacy of low-dose, catheter-directed ultrasound-accelerated thrombolysis (USAT) on the reversal of RV dysfunction is unknown.

Use of biomarkers or echocardiography in pulmonary embolism: the Swiss Venous Thromboembolism Registry

Cardiac biomarkers and echocardiography for assessing right ventricular function are recommended to risk stratify patients with acute non-massive pulmonary embolism (PE), but it remains unclear if these tests are performed systematically in daily practice. Design and methods: Overall, 587 patients with acute non-massive PE from 18 hospitals were enrolled in the Swiss Venous Thromboembolism Registry (SWIVTER): 178 (30%) neither had a biomarker test nor an echocardiographic evaluation, 196 (34%) had a biomarker test only, 47 (8%) had an echocardiogram only and 166 (28%) had both tests.

Pulmonary artery pressure and cardiac function in children and adolescents after rapid ascent to 3,450 m

High-altitude destinations are visited by increasing numbers of children and adolescents. High-altitude hypoxia triggers pulmonary hypertension that in turn may have adverse effects on cardiac function and may induce life-threatening high-altitude pulmonary edema (HAPE), but there are limited data in this young population. We, therefore, assessed in 118 nonacclimatized healthy children and adolescents (mean ± SD; age: 11 ± 2 yr) the effects of rapid ascent to high altitude on pulmonary artery pressure and right and left ventricular function by echocardiography. Pulmonary artery pressure was estimated by measuring the systolic right ventricular to right atrial pressure gradient. The echocardiography was performed at low altitude and 40 h after rapid ascent to 3,450 m. Pulmonary artery pressure was more than twofold higher at high than at low altitude (35 ± 11 vs. 16 ± 3 mmHg; P < 0.0001), and there existed a wide variability of pulmonary artery pressure at high altitude with an estimated upper 95% limit of 52 mmHg. Moreover, pulmonary artery pressure and its altitude-induced increase were inversely related to age, resulting in an almost twofold larger increase in the 6- to 9- than in the 14- to 16-yr-old participants (24 ± 12 vs. 13 ± 8 mmHg; P = 0.004). Even in children with the most severe altitude-induced pulmonary hypertension, right ventricular systolic function did not decrease, but increased, and none of the children developed HAPE. HAPE appears to be a rare event in this young population after rapid ascent to this altitude at which major tourist destinations are located.

Long-term cardiac remodeling and arrhythmias in nonelite marathon runners

Long-term endurance sports are associated with atrial remodeling and atrial arrhythmias. More importantly, high-level endurance training may promote right ventricular (RV) dysfunction and complex ventricular arrhythmias. We investigated the long-term consequences of marathon running on cardiac remodeling as a potential substrate for arrhythmias with a focus on the right heart. We invited runners of the 2010 Grand Prix of Bern, a 10-mile race. Of 873 marathon and nonmarathon runners who applied, 122 (61 women) entered the final analysis. Subjects were stratified according to former marathon participations: control group (nonmarathon runners, n = 34), group 1 (1 marathon to 5 marathons, mean 2.7, n = 46), and group 2 (≥6 marathons, mean 12.8, n = 42). Mean age was 42 ± 7 years. Results were adjusted for gender, age, and lifetime training hours. Right and left atrial sizes increased with marathon participations. In group 2, right and left atrial enlargements were present in 60% and 74% of athletes, respectively. RV and left ventricular (LV) dimensions showed no differences among groups, and RV or LV dilatation was present in only 2.4% or 4.3% of marathon runners, respectively. In multiple linear regression analysis, marathon participation was an independent predictor of right and left atrial sizes but had no effect on RV and LV dimensions and function. Atrial and ventricular ectopic complexes during 24-hour Holter monitoring were low and equally distributed among groups. In conclusion, in nonelite athletes, marathon running was not associated with RV enlargement, dysfunction, or ventricular ectopy. Marathon running promoted biatrial remodeling.

Outcome in adult patients after arterial switch operation for transposition of the great arteries

BACKGROUND: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist. METHODS AND RESULTS: We studied 145 adult patients (age>16, median 25years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (>45mm in 3 patients, maximal-diameter 49mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7-5.4, P<0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood. CONCLUSIONS: Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.

Leakage of the arterial prosthesis of an Impella RVAD

Seromas occurring around a vascular graft are a rare complication. We report a life-threatening plasma leakage that occurred through the polytetrafluoroethylene vascular prosthesis of an Impella right ventricular assist device (Impella RD [Impella Cardiosystems GmbH, Aachen, Germany]) implanted in a 62-year-old patient with acute right ventricular failure after cardiac transplantation. The leakage became progressively massive. Weaning the patient from the right ventricular assist device was not possible. The prosthesis was thus wrapped within a pericardial patch to contain the leakage. Three days later the patient could be successfully weaned and the pump was removed. The clinical evolution was favorable.

Central venous pulse pressure analysis using an R-synchronized pressure measurement system

Objective. The information derived from central venous catheters is underused. We developed an EKG-R synchronization and averaging system to obtained distinct CVP waveforms and analyzed components of these. Methods. Twenty-five paralyzed surgical patients undergoing CVP monitoring under mechanical ventilation were studied. CVP and EKG signals were analyzed employing our system, the mean CVP and CVP at end-diastole during expiration were compared, and CVP waveform components were measured using this system. Results. CVP waveforms were clearly visualized in all patients. They showed the a peak to be 1.8+/- 0.7 mmHg, which was the highest of three peaks, and the x trough to be lower than the y trough (-1.6+/- 0.7mmHgand-0.9+/- 0.5mmHg, respectively), withameanpulsepressureof3.4mmHg.ThedifferencebetweenthemeanCVPandCVPatend-diastoleduringexpirationwas0.58+/- 0.81 mmHg. Conclusions. The mean CVP can be used as an index of right ventricular preload in patients under mechanical ventilation with regular sinus rhythm. Our newly developed system is useful for clinical monitoring and for education in circulatory physiology.

Prostaglandin E1 testing in heart failure-associated pulmonary hypertension enables transplantation: the PROPHET study

BACKGROUND: Elevated pulmonary vascular resistance (PVR) is relevant to prognosis of congestive heart failure and heart transplantation. Proof of reversibility by pharmacologic testing in potential transplantation candidates is important because it indicates a reduced probability of right ventricular failure or death in the early post-transplant period. This study aimed to clarify the possible extent of acute reversibility of elevated PVR in a large, consecutive cohort of heart transplant candidates. METHODS: This study included 208 consecutive patients (age 52 +/- 10 years, 89% men and 11% women, ejection fraction 21 +/- 9%, Vo2max 12.6 +/- 4.2 ml/kg/min) being evaluated for heart transplantation in 7 transplant centers in Germany and Switzerland. Testing was performed with increasing intravenous doses of prostaglandin E1 (PGE1; average maximum dose 173 +/- 115 ng/kg/min for at least 10 minutes) in 92 patients exhibiting a baseline PVR of > 2.5 Wood units (WU) and/or a transpulmonary gradient (TPG) of > 12 mm Hg. RESULTS: PGE1 testing lowered PVR from 4.1 +/- 2.0 to 2.1 +/- 1.1 WU (p < 0.01), increased cardiac output from 3.8 +/- 1.0 to 5.0 +/- 1.5 liters/min (p < 0.01), and decreased TPG from 14 +/- 4 to 10 +/- 3 mm Hg (p < 0.01), mean pulmonary artery pressure (PAM) from 39 +/- 9 to 29 +/- 9 mm Hg (p < 0.01) and mean pulmonary capillary wedge pressure (PCWP) from 24 +/- 7 to 19 +/- 9 mm Hg (p < 0.01). Mean aortic pressure (MAP) decreased to 85% and systemic vascular resistance (SVR) to 65% of baseline values (p < 0.01). Symptomatic systemic hypotension was not observed. For the whole population the percentage of patients with PVR > 2.5 WU was reduced from 44.2% to 10.5% with PGE1. PVR decreased in each patient; only 2 patients (1%) remained ineligible for listing because of a final PVR of > 4.0 WU. TPG, ejection fraction and male gender were independent predictors of reversibility of PVR. CONCLUSIONS: Elevated PVR in heart transplant candidates is highly reversible and can be normalized during acute pharmacologic testing with PGE1.