Pregnancy and newborn outcomes in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected. RESULTS: Sixty pregnancies in twenty-three patients were identified between 1968 and 2016. Only two major non-fatal cardiac events (one sustained non-documented tachycardia and one ventricular tachycardia) were recorded during pregnancy in two different mothers (3% of pregnancies, 9% of mothers). None occurred during delivery or in the postpartum period. No mother developed heart failure. Beta-blocker therapy during pregnancy (n=15) was associated with lower birthweight (2730 vs 3400g, p=0.004). Only two preterm deliveries occurred, unrelated to cardiac condition. Caesarean section was performed in 13% of cases. Premature sudden-death occurred in 10% (n=5) of children before 25years-old including two in the first year of life. CONCLUSION: ARVC/D is associated with a low rate of major cardiac events during pregnancy and vaginal delivery appears safe. The risk of sustained ventricular arrhythmia seems poorly predictable and supports the continuation of beta-blockers during pregnancy. Major cardiac events were frequent in childhood, justifying close cardiac monitoring.

Respective role of surface electrocardiogram and His bundle recordings to assess the risk of atrioventricular block after transcatheter aortic valve replacement.

BACKGROUND: Atrioventricular block (AVB) is common after transcatheter aortic valve replacement (TAVR) and permanent pacemaker (PPM) implantation is needed in up to 30% of patients. Main predictors of long term AVB are electrocardiographic. The purpose of this study is to assess the prognostic value of serial HV intervals measured before and after TAVR to shorten the timing of PPM implantation. METHODS: His bundle recordings were performed before (HV1), immediately after TAVR (HV2) and at day 2 for Edwards Sapien (ES) and 5 for Medtronic CoreValve (CV) (HV3). PPM indications were high degree AVB before day 5 or prolonged HV interval ≥80ms at the last recording. High degree AVB after discharge was evaluated from the pacemaker memories and ECG at 1 and 6months. RESULTS: Data were obtained in 84 patients (33% CV and 67% ES). HV values were not associated with early or late AVB. PPM were implanted in 27 patients (34%) for documented AVB (n=17, 24%), prolonged HV interval (n=9) or sick sinus syndrome (n=1). Persistent complete AVB during the procedure and postoperative high degree AVB were the only perioperative factors associated with further long term occurrence of high degree AVB (p=0.001 and p<0.001). On multivariate analysis, only postoperative high degree AVB was significant (p=0.001). CONCLUSION: Pre- and post-operative HV measurements were not correlated with late AVB after TAVR. Perioperative persistent complete AVB and postoperative high degree AVB are the only factors to predict late AVB and should be considered for the decision of PPM implantation.

[Mycoplasma pneumoniae myopericarditis in children]. / Myopéricardite à Mycoplasma pneumoniae chez l'enfant.

Mycoplasma pneumoniae myocarditis is a rare condition, potentially very serious and seldom described in children. It is classically attributed to direct invasion or to an indirect immunological mechanism. The authors report the case of a 10 year old boy with myopericarditis, proved by authentic seroconversion, complicating congenital mitro-aortic valvular disease. In this case, the spectacular response to steroid therapy was in favour of an indirect immunological causal mechanism of the left ventricular dysfunction and pericardial involvement.

[Descriptive study of the patients admitted to an intensive care unit during the heat wave of August 2003 in France]. / Etude descriptive des patients admis en réanimation pour coup de chaleur au cours de la canicule d'août 2003.

INTRODUCTION: Several thousands of deaths were attributed to heat stroke during August 2003 in France. To date, only a very few studies have analyzed the prognosis in the intensive care unit (ICU) of the most severely hyperthermic patients. METHOD: Descriptive observational study of the patients admitted to the intensive care unit at the Lariboisière hospital in Paris, for heat stroke defined by an elevated core body temperature above 40 degrees C with central nervous system dysfunction, in the absence of other etiologies explaining the hyperthermia. RESULTS: In the Lariboisière hospital, an elevation in the ICU (+143%) and hospital mortality rate (+191%) were registered during August 2003, in comparison with August 2002. Fifteen patients (10 men, 5 women, median age: 57 years) were admitted to the ICU for heat stroke between the 4th and 14th of August 2003. Seven of them (47%) died. On admission, the occurrence of a pre-hospital cardiac arrest, the presence of coagulation abnormalities (reduction in prothrombin time and in platelet count) or of an elevation in plasma lactate concentration were significantly associated with the risk of death in the ICU. Conversely, age, body temperature, coma depth on admission and convulsions were not predictive of death. Neurological after effects (cerebellar syndrome, polyneuropathy and residual brain damage) were noted in 50% of the survivors. DISCUSSION: Although it is possible that heat alone precipitated the death of very sick people, our study clearly showed that young and valid patients died of heat stroke and suggests the possible increase in the 2003 death rate secondary to the heat wave. Moreover, it is still difficult at the moment to really appreciate the long-term consequences for survivors who presented serious neurological after effects. CONCLUSION: The August 2003 heat wave resulted in an elevation of the hospital and ICU death rates in the Lariboisière hospital in Paris. Despite adequate cooling and supportive therapies, the mortality of patients admitted to the ICU for heat stroke remained elevated and the neurological after effects severe. These preliminary results should be confirmed by larger cohort studies.

[Chylous effusion and surgical treatment of diaphragmatic hernia in 2 newborn infants]. / Epanchement chyleux après cure chirurgicale d'une hernie diaphragmatique chez 2 nouveau-nés.

BACKGROUND. Post-operative chylous effusion is rare in infants. It may result from venous thrombosis, thoracic duct injury or lymphatic vessel obstruction. This paper describes 2 cases of diaphragm hernia with post-operative chylous effusion. CASE REPORTS Case 1: A newborn developed chylous ascites two weeks after repair of a left posterolateral diaphragm hernia. Abdominal paracentesis showed milky fluid containing numerous lymphocytes (98%). Ascites was rapidly reduced by feeding with medium-chain triglyceride-rich (MCT) formula. Case 2: A chylothorax was seen 7 days after surgery to repair a left posterolateral diaphragm hernia. Thoracocentesis showed serous fluid containing numerous lymphocytes (93%). The chylothorax was resolved after two thoracocenteses, parenteral nutrition and feeding with MCT formula. CONCLUSION. These cases are the third report of chylothorax and the second report of chylous ascites after surgical repair of a diaphragm hernia. The exact cause of the chylous effusion is not clear.

[Rescue from sudden death syndrome and allergy to milk proteins (apropos of a case)]. / Syndrome de rescapé de mort subite et allergie aux protéines du lait (à propos d'une observation).

Allergy to milk proteins may be responsible for the syndrome of unexplained, sudden death of infants, but well-documented cases are rare. The authors report an observation where clinical history and complementary investigations support the incrimination of this factor.