Fetal lung lesions: a spectrum of disease. New classification based on pathogenesis, two-dimensional and color Doppler ultrasound.
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Bronchopulmonary Sequestration↗
Biomedical subjects
J Hegesh
Publications and source records attributed to J Hegesh.
Fetal lung dysplasia: clinical outcome based on a new classification system.
OBJECTIVE: To evaluate the clinical application of a new classification system of fetal lung anomalies. METHODS: Forty fetal diagnoses of lung lesions were analyzed according to our proposed classification system in which each lung component is considered using two-dimensional ultrasound and color and power Doppler technology. Medical files, natural history and neonatal follow-up were recorded. RESULTS: Type I dysplasia: Four cases of agenesis of the lung were diagnosed, three with right lung agenesis and one with left lung agenesis. Three of the four patients elected to undergo termination of pregnancy (TOP). The surviving fetus was diagnosed with scimitar syndrome and postnatal embolization of the aberrant vessel was performed. Type II dysplasia: One case of normal lung with abnormal systemic feeding artery was diagnosed with normal neonatal outcome. Type III dysplasia: Abnormal lung with abnormal vascularity was found in 14 cases, presenting in most cases as echogenic lung masses. Seven were supradiaphragmatic, six subdiaphragmatic and one case was of undetermined position. All 14 fetuses showed an aberrant systemic artery emerging from the aorta. Abnormal venous drainage could be identified in only five (36%) of the fetuses: three had prominent azygos vein, one showed drainage to the inferior vena cava and one had multiple intrapulmonary veins forming a huge arteriovenous (A-V) shunt. Two cases in this group underwent TOP, the case with A-V shunt following development of hydrops, and one on maternal request. The remaining 12 fetuses (86%) survived and were alive and well at the time of writing; only one of them needed immediate postnatal embolization of the bilateral aberrant feeding arteries. Type IV dysplasia: Abnormal lung with no vascular abnormality was diagnosed in 20 fetuses. In this group there was one case of intrauterine fetal death, two patients underwent TOP, one complicated with hydrops and one on maternal request. The survival rate in this group was 85%. Only two cases needed immediate surgical repair. Type V miscellaneous dysplasia: One fetus demonstrated echogenic lung with split notochord syndrome and survived. CONCLUSIONS: Congenital bronchopulmonary and related vascular anomalies can be categorized using the new classification system. This new approach enabled prenatal evaluation of each lung component and facilitated cogent management of the fetus with congenital lung dysplasia.
Blood Vessels↗
Antenatal sonographic findings of right pulmonary agenesis with ipsilateral microtia: a possible new laterality association.
Right pulmonary agenesis is a rare congenital malformation which results in secondary dextrocardia in situs solitus. Ipsilateral microtia in this context composes a laterality syndrome. The prenatal sonographic findings of this abnormality have not been previously reported. We describe the association of dextrocardia in situs solitus, intact diaphragm and right microtia. This was sonographically diagnosed at mid-gestation in an euploid fetus. Surgical evacuation of the pregnancy confirmed the external malformation. Laterality association should be assessed in a fetus with sonographic findings of pulmonary agenesis. The differential diagnosis and updated literature review is presented.
Adult↗
Fetal aortic arch measurements between 14 and 38 weeks' gestation: in-utero ultrasonographic study.
OBJECTIVE: To establish in-utero reference ranges for fetal transverse aortic arch diameter (TAD) and distal aortic isthmus diameter (DAID) using high-resolution ultrasound techniques. DESIGN: A prospective, cross-sectional study was performed on 125 normal singleton pregnancies between 14 and 38 weeks' gestation. Transverse and diameter and DAID were measured by transvaginal ultrasonography until 17 weeks' gestation, and by abdominal ultrasound between 18 and 38 weeks' gestation. RESULTS: Transverse arch diameter as a function of gestational age was expressed by the regression equation TAD = -1.17 + 0.169 X GA, and DAID = -1.39 + 0.189 X GA; TAD and DAID are transverse aortic and distal aortic isthmus diameters expressed in millimeters and GA is gestational age in weeks. The correlation r = 0.924 and 0.938 was found to be highly statistically significant (P < 0.001) for TAD and DAID. The normal mean of TAD and DAID per week and the 95% prediction limits were also defined. CONCLUSIONS: The normative data established by us may be helpful in the prenatal diagnosis of congenital heart defects, including abnormal growth of the aortic arch.
Adult↗
Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification.
OBJECTIVES: Anomalies of the fetal venous system are poorly documented and their pathogenesis is not well understood. The present study was undertaken to review the spectrum of fetal central veins and umbilico-portal system anomalies, and to propose a classification system. METHODS: A 7-year restrospective survey was conducted. RESULTS: Nineteen fetuses showed abnormal connection between central veins and the fetal heart. Three fetuses showed abnormal connections of the cardinal veins, two of which had interruption of the inferior vena cava, and one had isolated persistent left superior vena cava. Anomalies of pulmonary veins were seen in four fetuses: in two with asplenia syndrome, a vertical confluent pulmonary artery was observed. In a further two cases total anomalous pulmonary venous connections were found. Abnormalities of the umbilical vein (UV) were seen in 10 cases; seven had persistent right UV, and three had a spectrum of anomalies: One had abnormal connections of the UV to the left iliac vein associated with agenesis of the ductus venosus (DV) and hydrops fetalis. One case showed in utero occlusion of the DV by echogenic foci that resulted in a persistent left proximal UV and porto-systemic shunt. One case had obliteration of the DV secondary to in utero fetal hepatic fibrosis. Abnormalities of the vitelline veins or portal system were demonstrated in two cases. One had a left porto-systemic shunt which resolved spontaneously at 3 months of age, and one had secondary partial occlusion of the left portal system with liver echogenicities and direct communication of the UV with the right atrium. None of the 19 cases had an abnormal karyotype or evidence of in utero infection. CONCLUSIONS: Detection of various fetal vein anomalies in utero is feasible. The anomalies vary according to embryologic precursors or etiology. Two major mechanisms seem to be involved in the genesis of fetal vein anomalies: in most cases primary maldevelopment of the venous system occurs, while in the minority secondary anomalies from possible thromboembolic events or systemic disease may play a role.
Abnormalities, Multiple↗
Constrictive pericarditis presented by generalized edema (anasarca).
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Atrial Natriuretic Factor↗
Left superior vena cava to the left atrium: do we have to change the traditional approach?
Left superior vena cava (LSVC) to the left atrium is a rare congenital cardiac complex, which may appear as an isolated anomaly, or as part of more complex cardiac anomalies. Traditionally, an intraatrial baffle was the preferred surgical technique. Although this technique has proved reliable and successful, acute ligation and extracardiac repair are simpler and easier solutions, requiring less myocardial ischemic time. We present 3 patients who underwent simple ligation and discuss the literature for other extracardiac options of surgical repair. Our patients had short transient congestion in the left upper part of their body that resolved completely after a few weeks, without further complications. We believe that either acute ligation or extracardiac repair is a much simpler yet effective solution to divert the left caval flow to the lesser circulation.
Adult↗
[Transcatheter closure of atrial septal defects: initial clinical applications].
The incidence of congenital heart defects is 0.8% of liveborn infants. Of these 13% are ostium secundum atrial septal defects (ASD) which can be successfully repaired by open heart surgery with less than 1% mortality. However, morbidity associated with cardiac surgery is universal. Transcatheter closure of ASDs was introduced 2 decades ago to decrease surgical complications, hospital stay and cost. Few devices have undergone clinical trials but all of them have been associated with instances of failure and complications. The most important mechanism for acute failure was selection of cases with too large a defect or selection of a defective device. In the past decade great progress has been made with the aid of transesophageal echocardiography (TEE) and modifications of the device. We present our experience in our first 5 patients. They ranged in age from 4 to 27 years; the ASD diameter was 11-15 mm and the device diameter was 17-33 mm. All transcatheter closures were performed on the same day without any complications, and all patients were discharged home after 2 days of hospitalization. We conclude that transcatheter occlusion of ASDs up to 15 mm is feasible, relatively safe and effective. This transcatheter method appears to be a viable alternative to surgery for some patients with secundum atrial septal defects.
Adolescent↗
Congenital heart defects: natural course and in utero development.
BACKGROUND: Most congenital heart defects (CHDs) are diagnosed on targeted prenatal transvaginal (TVS) or transabdominal (TAS) ultrasonography during the early second trimester or at midgestation. Nevertheless, delayed diagnosis in some cardiac malformations still remains despite detailed echocardiographic examination. The present study was conducted to evaluate the evolution of fetal cardiac anomalies and assess their development in utero. METHODS AND RESULTS: We retrospectively reviewed 22,050 pregnant women who were divided into two groups: 6924 who had initial TVSs at 13 to 16 weeks' gestation, followed by TASs at 20 to 22 weeks, and 15,126 who had initial TASs at 20 to 22 weeks. Both groups were subsequently examined in their third trimester. All newborns were examined by certified pediatricians. CHD was diagnosed in 168 babies: 66 in group A and 102 in group B. In group A, 42 malformations (64%) were detected at the first TVS examination, and 11 (17%) were found during the subsequent TAS. Three additional anomalies (4%) were found during the third trimester, and 10 malformations (15%) were detected postnatally. In group B, 80 malformations (78%) were detected in the initial examination at midtrimester, and an additional 7 (7%) were found in the third trimester, whereas 15 (15%) were diagnosed postnatally. The 10 anomalies (group A, n=3; group B, n=7) that were detected only during the third trimester comprised aortic stenosis (n=2), cardiac rhabdomyoma (n=2), subaortic stenosis (n=1), tetralogy of Fallot (n= 1), aortic coarctation (n=1), sealed foramen ovale (n=1), ventricular septal defects (n=1), and hypertrophic cardiomyopathy (n=1). CONCLUSIONS: Although most fetal cardiac anomalies are detectable early in gestation, some may evolve in utero at different stages of pregnancy.
Adolescent↗
Congenital giant left atrial aneurysm in an infant.
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Diagnosis, Differential↗
Iodine and hypothyroidism in neonates with congenital heart disease.
AIM: To evaluate the influence of the intravenous injection of iodine during cardiac catheterisation, and of topical iodine antiseptics during surgical procedures, on thyroid function in full term neonates. METHODS: Twenty one full term infants with major cardiac anomalies who survived for more than a month were studied. Thyroxine and thyrotropin concentrations were measured (by radioimmunoassay) before each procedure, 24 hours after the procedure, and every week thereafter until the age of 1 month or until normal. Thyroxine values less than 64.4 nmol/l were considered low, while thyrotropin values greater than 30 mU/l were considered high. RESULTS: Thyroid function tests before iodine exposure were within normal limits in all infants. Following catheterisation or surgery six infants had raised thyrotropin concentrations; three had low thyroxine concentrations. Two of those infants were treated with L-thyroxine. CONCLUSION: Iodine exposure during cardiac catheterisation or surgery may induce transient hypothyroidism in term infants.
Anti-Infective Agents, Local↗
Prenatal diagnosis of vein of Galen aneurysmal malformation: report of two cases with proposal for prognostic indices.
Vein of Galen aneurysmal malformation (VGAM) is a rare, intracranial vascular anomaly that, until recently, has usually been diagnosed postnatally. Today, however, with the advances in high-resolution ultrasonography and colour Doppler, prenatal diagnosis is relatively easy. Due to novel intravascular embolization techniques, the prognosis of neonates with VGAM has markedly improved. A healthy infant with normal neurodevelopmental and cardiovascular status is now a reality. For the best outcome, however, careful planning of the appropriate time, mode, and place of delivery should be undertaken. To achieve this goal, in utero prognostic factors should be determined. This report illustrates, for the first time, prenatal ultrasonographic indices that may predict the outcome in two cases with a prenatal diagnosis of VGAM. The indices included mapping of intracranial feeding arteries, assessment of the width of the straight sinus, assessment and measurement of flow in the straight sinus, existence of 'steal' retrograde aortic flow, and the appearance of high-output cardiac state. By using these prenatal ultrasonographic parameters, fetal outcome may be predicted and appropriate management decided upon.
Adult↗
In utero ultrasonographic diagnosis of an aberrant umbilical vein associated with fetal hepatic hyperechogenicity.
Intra-hepatic abnormalities of the fetal umbilical venous system are poorly documented and clinically not well understood. A case of routine ultrasound examination at 23 weeks' gestation demonstrating foci of hepatic hyperechogenicity and cardiomegaly is presented. Colour Doppler detected absence of flow in the ductus venosus and markedly increased blood flow through an aberrant channel connecting the umbilical vein with the right atrium. The pregnancy was terminated and anomalous venous drainage of the umbilical vein into an enlarged hepatic vein was found, as well as hepatic congestion and focal hepatic necrosis and calcifications. Incidental findings of fetal hepatic hyperechogenicities require colour Doppler investigation of the intra- and extra-hepatic venous systems. We propose that a thrombo-embolic mechanism may be involved in the pathogenesis of these lesions.
Adult↗
In utero congestive heart failure due to maternal indomethacin treatment for polyhydramnios and premature labour in a fetus with antenatal closure of the foramen ovale.
A case of severe fetal congestive heart failure due to occlusion of the ductus arteriosus in a mother treated with indomethacin for polyhydramnios and premature contractions is described. Closure of the fetal foramen ovale that escaped detection by prenatal echocardiography was later demonstrated at neonatal autopsy. This case suggests that indomethacin treatment in a ductus-dependent fetus may be hazardous. Therefore, careful surveillance of the fetus exposed to indomethacin in utero is warranted.
Adult↗
Antenatal sonographic findings associated with scimitar syndrome.
Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs solitus, due to right pulmonary hypoplasia. The prenatal sonographic findings of this abnormality have not been previously described. We describe early sonographic findings in a fetus that included isolated cardiac dextroposition with normal abdominal situs, hydramnios and mild narrowing of the right pulmonary artery. Postnatally, the infant developed heart failure, and a right hypoplastic lung was found. Catheterization revealed collateral supply to the right lung arising from the descending aorta and anomalous drainage of right pulmonary veins to the inferior vena cava. The diagnosis of scimitar syndrome was therefore established. A coil embolization of arterial collaterals to the right lung was performed. Scimitar syndrome should be considered in a fetus with the sonographic findings of a right shift of the mediastinal structures, an intact diaphragm and narrow right pulmonary artery. This may allow early neonatal stabilization and treatment.
Adult↗
Adenosine for the treatment of paroxysmal supraventricular tachycardia in full-term and preterm newborn infants.
Adenosine has been approved for intravenous use for paroxysmal supraventricular tachycardias (SVT) in adults and children. However, effectiveness and safety of intravenous adenosine in preterm infants are not well established. Thirteen episodes of SVT in three preterm and two full-term neonates were treated with intravenous adenosine. All had narrow QRS tachycardia at 230 to 260 beats/min. Adenosine prepared as a sterile 1 mg/mL solution was given as an intravenous bolus starting at 0.05 mg/kg, and increased by 0.05 mg/kg until tachycardia was terminated. Termination of tachycardia was achieved within 12 to 25 seconds in all patients. In one, termination of SVT was followed by temporary suppression of the sinus node, followed by resumption of normal sinus rhythm. No other side effects were noted. Adenosine is a safe and effective agent for treating preterm infants with SVT. However, further investigation of adenosine in this group of patients is warranted.
Adenosine↗
End-tidal CO2 levels are a reliable indicator of band tightness in pulmonary artery banding.
BACKGROUND: Monitoring of end-tidal CO2 levels, performed routinely nowadays in most operating rooms, is obligatory in our hospital for all anesthesia patients. Levels are dependent on pulmonary blood flow, ventilation, and CO2 content of blood. When ventilation is kept constant, the end-tidal CO2 closely follows pulmonary blood flow. METHODS: Reduction of end-tidal CO2 in the expired air was used to adjust tightness of the pulmonary band in 10 patients with complex cardiac anomalies, all including ventricular septal defect, who underwent pulmonary artery banding. Other parameters were systemic blood pressures and distal pulmonary artery pressures. RESULTS: There were no operative deaths. Average reduction was 3.8 mm Hg (range, 2 to 10 mm Hg; p < 0.001 by paired t test), average increase in systolic blood pressure was 14 mm Hg (range, 4 to 20 mm Hg; p < 0.03 by Wilcoxon sign rank test), distal pulmonary artery pressure was reduced from 56 mm Hg (range, 37 to 79 mm Hg) to 29 mm Hg (range, 20 to 38 mm Hg; p < 0.03 by t test), and postoperative pulmonary artery to systemic pressure ratio averaged 0.36 mm Hg (range, 0.24 to 0.49 mm Hg, difference from preoperative value, p < 0.06). CONCLUSIONS: End-tidal CO2 tension is a simple and convenient, yet highly reliable parameter for adjusting pulmonary artery band tightness.
Carbon Dioxide↗
Successful radiofrequency ablation in a 3-month-old baby with permanent junctional reciprocating tachycardia: a new era in the treatment of incessant life-threatening arrhythmias in infants.
We report our experience in treating a 3-month-old baby with accessory atrioventricular pathway by radiofrequency (RF) ablation. The baby was discovered to have incessant supraventricular tachycardia at 4 weeks of age. At that time, two-dimensional echocardiography showed dilated cardiomyopathy. Multiple direct current shocks and multipharmaceutical treatment failed to restore sinus rhythm. Electrophysiology study showed the presence of posteroseptal accessory pathway. Immediately after delivery of two RF pulses, successful ablation of the accessory pathway and complete atrioventricular (AV) block with good junctional escape rhythm were observed. Normal rhythm and AV conduction subsequently returned and all medical treatment was discontinued. To our best knowledge, this is the first case of successful treatment with RF ablation in early infancy.
Catheter Ablation↗