Deadly vasospasm.

A patient with Prinzmetal's variant angina (PVA) developed a cardiac arrest due to coronary vasospasm and subsequent myocardial infarction. After resuscitation postanoxic brain injury was diagnosed. After an initial improvement of consciousness he deteriorated rapidly on the seventh day after admission due to severe brain ischaemia apparently caused by cerebral vasospasm, until ultimately brain death was diagnosed. To our knowledge, the association between PVA and cerebral vasospasm has never been described. The combination suggests that this patient had a generalized vasospastic disorder.

[Two patients with Eisenmenger syndrome treated with novel agents that target vasodilation of the pulmonary capillary bed]. / Twee patiënten met het syndroom van Eisenmenger behandeld met nieuwe medicijnen gericht op vasodilatatie van het longvaatbed.

Two women with Eisenmenger syndrome, aged 63 and 45 years, presented with different symptoms: the first patient had peripheral oedema, proteinuria, progressive fatigue and cyanosis and the other had increasing dyspnoea and blue lips. The first patient was successfully treated with diuretics but experienced a collum fracture that occurred after hypovolemic collapse caused by diuretic use. She was given sildenafil and underwent hip surgery with spinal anaesthesia 10 days later. In the following weeks, the patient was haemodynamically stable but then died suddenly; no autopsy was performed. The second patient was given oxygen therapy at home and bosentan. After 6 months the symptoms of dyspnoea resolved and her 6-minute walking distance increased from 453 to 512 m. The life expectancy of patients with congenital heart disorders such as Eisenmenger syndrome has improved dramatically, due in part to the efficacy of novel agents that inhibit endothelial-cell proliferation. With these advances, treatment of these patients is no longer restricted to tertiary-care centres. Therefore, community cardiologists, pulmonologists and internists should be aware of these congenital heart disorders and the available treatment options.

Arrhythmogenic right ventricular cardiomyopathy: asymptomatic to life threatening as illustrated by the cases of two sisters.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disorder of unknown cause that is characterised by fibrofatty replacement, primarily of the right ventricular myocardium, which can lead to life-threatening arrhythmias. It is a disease with a very diverse phenotype. In the present article we describe two sisters, each with a different manifestation of this disorder. The first patient died suddenly at the age of 18 during exercise. Her 17-year-old sister did not have any abnormalities at first cardiac consultation, but a few years later she met several diagnostic criteria for ARVC and an internal cardioverter defibrillator was implanted. Genetic analysis identified a mutation in the plakophilin- 2 (PKP2) gene. Cardiac evaluation of a third sister did not reveal any abnormalities and no mutation in the PKP2 gene was found. Thus, ARVC can vary in its clinical presentation, not only between siblings but also in time. This raises difficulties for the physician for diagnosis, treatment and followup. It is important for the physician involved to consider this disease in patients with palpitations and syncope, especially when there is a family history of ARVC or unexplained sudden death. (Neth Heart J 2007;15:348-53.).

Cardiogenetic counselling in a non-university hospital.

BACKGROUND: Inherited heart disease is becoming a substantial part of everyday cardiology practice while genetic counselling still only takes place at university hospitals. In this study we review our seven-year experience with cardiogenetic counselling in a non-university hospital. METHODS: Retrospective analysis of patient records. RESULTS: A total number of 83 index patients were counselled. In 65 patients DNA tests were performed, resulting in 26 positive tests. In all patients with genotype confirmation of hereditary cardiovascular disease and in 32 families without a molecular diagnosis, family screening was advised. Out of 120 subsequently tested family members, 47 molecular genetic diagnoses were confirmed. CONCLUSION: Although the number of patients reviewed was small, our data show that cardiogenetic diseases are part of daily cardiology practice. We believe counselling should be performed in more general hospitals. This is an excellent opportunity for collaboration between university and nonuniversity hospitals, with immediate benefit for patients and their relatives. (Neth Heart J 2007;15:412-4.).

Transesophageal echocardiographic evaluation of left ventricular function during intraaortic balloon pump counterpulsation.

Transesophageal echocardiography was used to study the effects of intraaortic balloon pump counter pulsation (IABP) on left ventricular dimensions and function in 16 hypotensive patients after cardiac surgery. The short-axis cross section at midpapillary muscle level was used to determine systolic and diastolic dimensions. We found a significant decrease in end-systolic and end-diastolic area and increase in fractional area change during IABP-supported circulation. Regional area ejection fraction analysis demonstrated an improvement during IABP of impaired (particularly severely impaired) function at baseline. We conclude that both regional and global left ventricular function improve by the use of IABP in conjunction with a decrease of left ventricular size.

Transesophageal echocardiography in hypotensive patients after cardiac operations. Comparison with hemodynamic parameters.

Because it is sometimes difficult to determine the cause of hypotension in patients after cardiac operations, we assessed the value of transesophageal echocardiography in this respect, and we studied 60 consecutive patients who had hypotension despite positive inotropic medication and, in some patients, mechanical support. Echocardiographic diagnoses were compared with diagnoses based on hemodynamic parameters. Follow-up examinations were completed in all patients to confirm the final diagnoses. Echocardiographic signs of hypovolemia were present in 14 patients, tamponade in six, left ventricular failure in 16, right ventricular failure in 11, and biventricular failure in eight. Echocardiographic examination proved to be inconclusive in five patients. Comparison with hemodynamic parameters showed agreement on diagnoses (hypovolemia versus tamponade versus cardiac failure) in 30 patients (50%). Echocardiography correctly identified two patients with tamponade and six with hypovolemia; these conditions were not suspected by standard hemodynamic data; in five patients unnecessary reoperation was prevented, although hemodynamic values were suggestive of tamponade. Echocardiography also identified subcategories of patients at high risk of death (those with signs of right ventricular and biventricular failure). These findings suggest that transesophageal echocardiography performed on patients after cardiac operations, at the bedside in the intensive care unit, can readily elucidate the cause of hypotension in the large majority of patients and is a valuable adjunct to hemodynamic evaluation in patient management. Furthermore, it appears to be possible to identify subcategories of high-risk patients, based on these echocardiographic findings.

Prognostic value of biventricular function in hypotensive patients after cardiac surgery as assessed by transesophageal echocardiography.

In patients after cardiac surgery, hypotension, defined as a mean arterial pressure less than 65 mmHg despite adequate filling pressures and positive inotropic medication, poses a problem. In addition, it is often difficult to determine whether these patients have suffered irreversible myocardial injury or if they are likely to recover. In this study, left and right ventricular function, as assessed by transesophageal echocardiography (TEE), was related to mortality both (1) quantitatively, using fractional area change (FAC), and (2) qualitatively, using a segmental wall motion analysis, which assigned a score to myocardial wall segments, in order to determine whether this technique can be used to predict survival. Mortality rate was very high in patients with biventricular and especially right ventricular failure (FAC less than 35%). Left and right ventricular wall motion abnormality indices were significantly better in survivors compared to nonsurvivors, but no distinct cut-off value could be determined. A wall motion index derived from only 6 segments at the mid-papillary muscle level was found to be as reliable as one based on 16 segments of the entire left ventricle. Thus, TEE provided information about the degree of left and right ventricular dysfunction by using a single cross-section at the papillary muscle level. It identified patients at high risk of death, ie, those with compromised right and biventricular function.

Transesophageal Echo-doppler evaluation of the hemodynamic effects of positive-pressure ventilation after coronary artery surgery.

Transesophageal echocardiography was used to extend knowledge about the impact of positive end-expiratory pressure (PEEP) during mechanical ventilation on right and left ventricular function and right ventricular impedance. At 20 cmH2O PEEP, a progressive increase of right ventricular end-diastolic area was seen (27%) that coincided with a reduction of early left ventricular filling velocity (25%) across the mitral valve, and a decrease of both pulmonary artery flow velocity (end-expiration 27% and end-inspiration 42%) and time-velocity index (end-inspiration 25%). As these changes were not accompanied by a change of the fractional area of contraction, the increase of the right ventricular diameter might be explained by right ventricular compensation due to an imbalance between augmented right ventricular impedance and reduced venous return.