Hypertrophic obstructive cardiomyopathy and pregnancy: anesthesiological observations and clinical series.

Hypertrophic obstructive cardiomyopathy represents a genetic disorder characterized by hypertrophy, usually asymmetrical, of the ventricular musculature at the base of the septum in the left ventricular efflux tract. Patients suffering from this disorder can be extremely sensitive to small alterations in ventricular volumes, arterial pressure, cardiac frequency and rhythm. This disorder is found in pregnancy with an incidence of 0.1-0.5% and, because of its gravity, represents a contraindication which is often absolute to pregnancy. Hemodynamic variations such as those found in pregnancy, labor and delivery have complex influences on hypertrophic cardiomyopathy. Our clinical series includes 2 pregnant patients suffering from hypertrophic obstructive cardiomyopathy who both underwent caesarian section in general anesthesia, the first due to the gravity of cardiac obstruction and the second due to the emergent need to proceed after the beginning of labor. The small number of clinical cases in the literature, especially in the last few years, clearly underlines the difficulty of defining both the most correct method for delivery and the most appropriate anesthesiological techniques. In accordance with the literature and our clinical experience, we can conclude that a carefully managed pregnancy can proceed without complications in patients with moderate obstruction and that a regional anesthesiological approach is also possible with careful hemodynamic monitoring. General anesthesia, however, remains the safest method and has fewer risks for patients with serious obstruction or with worsening of their clinical condition during pregnancy.

EXIT procedure in a twin gestation and review of the literature.

Prenatal diagnosis can show masses of the fetal neck, mouth, and face that can potentially cause respiratory distress at birth. To prevent such an emergency, the EXIT (ex utero intrapartum technique) is performed: it is the intrapartum intubation of the fetus at term while still connected to the placenta. The EXIT procedure was first performed in a case of cervical teratoma. Up to now a total of 34 cases are described, mostly cervical teratomas (13 cases), lymphangiomas (7), epignathus (3); babies' outcome has been successful in 25 of them, with one death related to the procedure. Among the reported cases we are aware of only one where EXIT was performed in a twin gestation, in which the normal twin was delivered first. In our case the normal fetus was posterior to the twin with cervical malformation, requiring us to work on the latter while the former was still in the uterus. After having safely secured the airway in twin A, twin B was prompt delivered with excellent general conditions. Our limited experience enlarges the possibility to perform this prenatal procedure even in "nonstandard" conditions, such as a twin gestation, and may prove useful to those who are going to deal with such issues.

[The EX-utero Intrapartum Technique (EXIT) procedure in Italy]. / Il parto ex-utero intrapartum technique (EXIT) in Italia.

Aim of the study was to present the first two Italian cases of C-section performed with the EXIT procedure (EX-utero Intrapartum Technique). Deliveries were performed at the Division of Obstetrics and Gynecology of the Hospital of Padua in cooperation with the Pediatric Surgery Department, both tertiary care centers. The first case was a twin with a huge neck mass (cystic hygroma) and the second a fetus with an oropharyngeal mass (epignathus). Airway patency could have been compromised at birth in both of them. EXIT procedure consists in securing the airway of the fetus partially delivered and still connected with the placenta. This technique leaves an intact feto-placental circulation and guarantees a normal fetal oxygenation while fetal airway patency is secured. Both the fetuses were successfully intubated and the C-section ended up in a short period of time without maternal and fetal complications. The EXIT technique, performed for the first time in 1989 and now in many centers abroad, can be considered a safe procedure as long as a multidisciplinary approach is carried out. The EXIT procedure is indicated whenever fetal airways can be compromised at birth, that is when oropharyngeal masses, laryngeal atresia, cystic hygroma and goiter are encountered during prenatal ultrasound.

[Hemolytic uremic syndrome in twin pregnancy at 32 weeks gestation with HELLP syndrome. Case report]. / Sindrome uremico emolitica in gravidanza gemellare alla 32(a) settimana con HELLp syndrome. Descrizione di un caso clinico.

Renal failure occurring in pregnancy or post partum is an unusual but well-described complication. Acute renal failure seems to be associated more often with HELLP syndrome rather than with pre-eclampsia or chronic hypertension. Probable overlapping of HELLP and hemolytic uremic syndrome in pregnancy or postpartum should be taken into consideration when treating pregnant women who show signs of proteinuria, hypertension, hematuria, increase of reticulocytes, decrease of haptoglobin with thrombocytopenia and microangiopathic hemolytic anemia. Our case refers to a 32 year old woman at 32 weeks gestation in twin pregnancy who presented with HELLP syndrome and renal failure. Immediately postpartum oliguria was noted and the laboratory analyses suggested the coexistence of HELLP and hemolytic uremic syndrome. In patients with gestosis and/or HELLP syndrome presenting oliguria combined with a decrease of hemoglobin level not due to intraoperative hematic leaks it is always necessary to ask for haptoglobin dosage. In treating hemolytic uremic syndrome it is very important to use a high dosage of plasma and sometimes plasmapheresis. HELLP syndrome contributes to various complications which are sometimes responsible for kidney or maternal mortality. In treating these patients early diagnosis combined with a specific treatment can considerably reduce kidney and maternal mortality.

High risk pregnancy in a patient with angioma of the corpus callosum.

Literature data indicate that rupture of arterovenous malformations is 4 times more frequent in pregnant women. The Authors present a case of cerebral hemangioma of the third medium anterior of the corpus callosum diagnosed in a patient at the 28th gestational week by Magnetic Resonance scan. The patient underwent elective caesarean section under peripheral anesthesia (peridural and spinal block by Bupivacaine) and delivered a male infant 4560 gr, 52 cm. The postoperative course was uneventful. The arterovenous malformation constituted an important anesthesiologic risk factor but the Authors conclude that caesarean section can be safely performed with a very careful anesthesiological time schedule and a reliable haemodynamic monitoring.

Cardiotocographic modifications during continuous epidural anaesthesia in labour.

Cardiotocographic variations certainly attributable to epidural anaesthesia were studied over the period 1972-1980 on 716 patients in labour. Transitory side-effects due to both direct and indirect action of local anaesthetics appeared in 1.4% of the cases, without, however, subsequently creating any particular foeto-newborn problem. On the basis of the experience acquired, it seems justifiable to affirm that the correct use of continuous epidural block, affected by very small doses of bupivacaine (10-20 mg per administration) is almost risk-free. The only inconvenience therefore that may sometimes arise would only be due to the relevation of a concealed cava occlusion when the maintenance of a left lateral position is not observed.