Pulmonary Branches Research Articles

Repair of persistent truncus arteriosus with interrupted aortic arch

The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.

Angiographic detection of the left anterior descending and the right coronary artery after fistulas into the pulmonary artery in a patient with rheumatic mitral stenosis

Coronary artery fistula is a rare heart defect found in approximately 0.2% of the adult population undergoing coronary angiography. The diagnosis is usually made by aortography and selective coronary angiography. We report here an adult patient with rheumatic mitral stenosis and left anterior descending coronary artery and pulmonary conus branch of right coronary artery–pulmonary artery fistulas detected by coronary angiography.

No increase in peak systolic velocity of the pulmonary veins over time: a potential marker for congenital lobar emphysema

No increase in peak systolic velocity of the pulmonary veins over time: a potential marker for congenital lobar emphysema

Aspergillus fumigatus Endocarditis in a Patient With a Biventricular Pacemaker

Aspergillus fumigatus endocarditis is one of the rarest and severest complications in cardiological patients. We describe a patient with an intracardial pacemaker who was diagnosed as having Aspergillus fumigatus endocarditis. Postmortem examination showed a large, Aspergillus-infected thrombus encased in the right ventricle, pulmonary trunk and main pulmonary branches.

Left Pulmonary Arterioplasty—Extended End-to-End Anastomosis

Surgical arterioplasty for left pulmonary branch stenosis often produces unsatisfactory results. We report a new operative approach involving a modification of end-to end anastomosis, providing a widely patent and nonredundant anastomosis.

Conditions leading to pediatric cardiology consultation in a tertiary academic hospital.

To determine the basis for cardiac consultations for pediatric patients in an academic hospital setting. The activities of the cardiology consultation service were tabulated for 12 months, from July 2001 to June 2002. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained by the consultation service, encounter forms submitted to the billing office, consultation notes maintained in a central file, and a departmental list of echocardiography studies. Patients who required clearance for noncardiac surgical procedures were generally evaluated in the cardiology clinic and not by the consultation service. Patient data were obtained from consultation and echocardiography reports and from hospital computer-based records for discharge summaries for inpatient admissions, emergency department encounter summaries, and laboratory reports. For each patient, consultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate admissions, or for different clinical concerns during a single admission. A total of 2071 consultations were performed for 1724 patients. The age at the time of consultation was 6.6 +/- 9.3 years (median: 1.2 years; range: 1 day to 60.6 years). A total of 1507 patients (87.4%) had a single consultation; 217 patients (12.6%) had multiple encounters, ranging from 2 to 9, accounting for 564 consultations (27.2%). Clinical concerns included murmurs (18.5%), cardiac function (12.7%), arrhythmias (12.7%), intercurrent illnesses among cardiac patients (11.3%), cyanosis (6.3%), syndromes (5.7%), chest pain (5.2%), syncope/dizziness (4.5%), subacute endocarditis (4.4%), follow-up evaluations of fetal diagnoses (4.3%), Kawasaki disease (3.4%), cor pulmonale (3%), recent cardiac surgery or catheterization (1.6%), cerebrovascular accidents (1.2%), and miscellaneous conditions. Four diagnoses accounted for 91% of murmur evaluations, ie, patent ductus arteriosus, ventricular septal defects, innocent murmurs, and pulmonary branch murmur of infancy. The most common murmur diagnosis in the neonatal intensive care unit was patent ductus arteriosus (68%), in the well-child nursery was ventricular septal defect (64%), and on the medical ward was innocent murmur (62%). The most common basis for evaluation of function was oncologic disease. Among patients evaluated for function, there were 3 new diagnoses of structural congenital heart disease, all involving neonates with aortic arch obstruction. Approximately two-thirds of arrhythmias were supraventricular in origin. The most common arrhythmias requiring treatment were supraventricular tachycardia and atrial flutter/fibrillation, the latter occurring mainly among older patients with structural heart disease. Diagnoses made with fetal echocardiography accounted for 14.3% of newborn consultations and included 83% of patients with cyanotic cardiac disease. Three syndromes accounted for 57% of consultations for this indication, ie, VACTERL association (vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal abnormality, and limb anomalies), trisomy 21, and infant of diabetic mother. Chest pain and syncope/dizziness were frequently evaluated in the emergency department and, in this setting, accounted for 13 and 10% of all evaluations and 19 and 25% of evaluations for new patients, respectively. For patients evaluated for chest pain, the most common basis was musculoskeletal/costochondritic (42%) or idiopathic (22%). There was a cardiac or pericardial basis in 11% of cases; these patients either had known heart disease associated with this complication or systemic symptoms, abnormal cardiac auscultatory findings, and electrocardiographic features of pericarditis. Syncope/dizziness most commonly had a vasovagal (50.5%) or orthostatic (24.7%) basis. There was a cardiac basis in 5.4% of cases; these patients were more likely to have symptoms associated with exercise. Although endocarditis was a frequent clinical concern (91 patients), only 3 cases were identified, involving 2 patients with structural heart disease and 1 neonate with an indwelling intracardiac catheter. Two other patients had central venous lines, intravascular thrombus, and fungemia. Kawasaki disease was the most common acquired condition leading to consultation. Cor pulmonale was most commonly screened among patients with congenital diaphragmatic hernia, chronic lung disease of prematurity, pneumonitis, reactive airway disease, or cystic fibrosis. Patients with recent cardiac surgery or cardiac catheterization typically had postpericardiotomy syndrome or complications associated with vascular access. Approximately 20% of cases of cerebrovascular accidents had a cardiac basis. Although a variety of conditions were assessed, some were encountered more frequently. Future educational curricula developed for cardiac training of pediatric residents should appropriately emphasize conditions necessitating consultation.

Prenatal Features of a Truncus Arteriosus With Pulmonary Atresia and Pulmonary Circulation Derived From the Ductus Arteriosus

truncus arteriosus is a solitary great artery arising from the base of the heart and supplying the systemic, pulmonary, and coronary circulation. A truncus arteriosus is a rare cardiac defect, accounting for less than 1% of structural heart defects.1 The single great artery usually arises above a malalignment ventricular septal defect (VSD) and straddles the ventricular septum. There is variability in the origins of the pulmonary branch and whether the aortic arch is interrupted. Van Praagh and Van Praagh1 suggested classifying trunci arteriosi with VSD into 4 types. In type I, a main pulmonary artery arises from the truncal root and bifurcates into the branch pulmonary arteries. In type II, the branch pulmonary arteries are separate from the truncal root. In type III, the left pulmonary artery is supplied by a collateral from the aortic arch and does not arise from the truncal root. In type IV, the aortic arch is interrupted. A truncus arteriosus may rarely occur without a VSD. Although prenatal diagnosis of a truncus arteriosus has been reported several times,2–4 to our knowledge, the rare variant with an atretic main pulmonary trunk and normal pulmonary circulation such as the case presented here has not been described prenatally. The objective of this report was to show abnormal fetal circulation associated with a truncus arteriosus with pulmonary atresia but with well-developed pulmonary circulation derived from retrograde flow through the ductus arteriosus.

A operação de Glenn bidirecional deve ser realizada com ou sem o auxílio de circulação extracorpórea?

OBJETIVO: Comparar os pacientes onde a operacao foi realizada com circulacao extracorporea (CEC) aqueles sem CEC, analisando suas caracteristicas e verificando se houve ou nao superioridade de alguma das tecnicas empregadas. METODO: Entre janeiro de 2002 e janeiro de 2004, 16 pacientes foram submetidos a operacao de Glenn bidirecional (GB), todos com defeitos cardiacos complexos. Quatorze eram do sexo feminino, com idade mediana de 19 meses. Os pacientes foram divididos em dois grupos: A (GB com CEC) e B (GB sem CEC), sendo sete do grupo A e nove do grupo B. Foram analisadas comparativamente as variaveis: sexo, idade, pressao arterial pulmonar media (PAPM), operacao realizada, tempo de CEC, pincamento da aorta, derivacao venoatrial, operacoes previas, internacao na unidade de terapia intensiva (UTI), hospitalizacao e complicacoes imediatas. RESULTADOS: A mediana da PAPM foi 13 mmHg. No grupo A, o tempo foi de CEC 91 ± 47 minutos (57-195 minutos) e o de isquemia miocardica 25 ± 33 minutos (0-80 minutos). Destes, quatro necessitaram de procedimento intracardiaco ou ampliacao de ramos pulmonares e tres, auxilio da CEC como suporte ventilatorio. No grupo B o tempo de desvio venoatrial foi de 21 ± 10 minutos (0-39 minutos). O tempo para extubacao 9 ± 13 horas, mediana 3 horas (1-43 horas). A permanencia UTI foi de 8 ± 12 dias, mediana 5 dias (1-50 dias). O periodo medio de internacao hospitalar foi de 12 ± 12 dias, mediana 7 dias (0-50 dias). Cinco pacientes haviam sido submetidos a operacao previa. Dois (12,5%) faleceram, sendo um em cada grupo. Nao houve complicacoes neurologicas, derrame pleural ou pericardico. Todas as variaveis estudadas entre os grupos nao apresentaram diferenca estatistica significativa. CONCLUSAO: Apesar da amostra relativamente pequena, o presente estudo sugere que a operacao de GB pode ser realizada com ou sem CEC com resultados semelhantes no que se refere a morbidade e mortalidade. Assim sendo, a operacao sem CEC pode ser empregada com seguranca quando os achados anatomicos forem apropriados e nao houver hipoxia grave.

Genetic basis of congenital heart disease

Although the care of infants and children with congenital heart defects has been revolutionized over the past 50 years, their underlying causes have been obscure. Recent progress, accelerated through the Human Genome Project, has resulted in the rapid identification of disease genes causing congenital heart defects. In this review, progress in identifying new congenital heart defect genes for specific lesions and in understanding their disease pathogenesis in the past year are detailed. Specifically, genes relevant for atrial and atrioventricular septal defects, patent ductus arteriosus, bicuspid aortic valve, and coarctation of the aorta as well as pulmonary valvar and branch stenosis are reviewed. The information in this review provides insights into the state-of-the-art knowledge about the molecular genetic causes of congenital heart defects. It suggests that DNA testing may become standard for many forms of congenital heart defects, improving clinicians' ability to anticipate complications for their patients and predict recurrence risk for families of children with congenital heart defects.

Tetralogía de Fallot con agenesia de válvula pulmonar en el recién nacido y lactante. Corrección completa

We report two patients, a newborn and a 7-month old infant, with tetralogy of Fallot and absent pulmonary valve syndrome. Both had severe obstruction at the level of the ring with aneurysmal pulmonary artery branches, which compressed and displaced the trachea and main bronchial tubes. The neonate required mechanical ventilation from birth. Treatment was aggressive in both patients with interventricular septum defect closure, arterioplasty of the branches and homograft in the infant, and resection of the truncus and pulmonary branches with posterior face suture of both branches associated with a valved conduit in orthotopic position in the neonate. We believe that early treatment avoids airway degeneration and right ventricle volume overload.

Resonancia magnética y gammagrafía de perfusión pulmonar en casos de tetralogía de Fallot operados

To evaluate pulmonary arteries in patients with tetralogy of Fallot following surgery with quantified lung perfusion scintigraphy and magnetic resonance imaging. From January 1985 to December 1999, 47 patients who underwent surgery between 1985 and 1999 were studied. To obtain values of normality, 45 infants with no pulmonary artery disease were assessed with lung perfusion scintigraphy (right lung flow: 54-61%, left lung flow: 38.7-46%) and magnetic resonance imaging axial view (right/left pulmonary artery branch diameter ratio: 1-1.1). Patients with stenosis underwent catheterization. Group 1: 27 patients with normal parameters. Group 2: nine patients with left pulmonary branch stenosis and irregularities in all parameters; left/right branch diameter ratio 0.51 and left lung perfusion 26.3 +/- 7.9%, r correlation ratio 65%, p < 0.005, left branch pressure gradient 34.4 +/- 17.9 mm Hg, rate-gradient r 89%, p < 0.001. Group 3: five patients with right pulmonary branch stenosis and irregularities in all parameters; right/left branch diameter ratio 0.52, reduced right lung perfusion 32 +/- 11%, with a stenotic branch gradient of 40 +/- 19 mm Hg., rate/gradient ratio r 72%, p < 0.005; gradient/perfusion ratio r 82%, p < 0.003. Group 4: Six patients with bilateral stenosis, reduced diameter in the stenotic area with normal perfusion in both lungs and bilateral gradient. Eighteen patients with stenosis underwent re-operation, with favorable outcome in 14. Magnetic resonance imaging and quantified lung perfusion scintigraphy provide useful information in the follow-up of tetralogy of Fallot.

Pulmonary vein dimensions and variation of branching pattern in patients with paroxysmal atrial fibrillation using magnetic resonance angiography.

Pulmonary veins are the most frequent origin of focal and paroxysmal atrial fibrillation. Although radiofrequency ablation has been attempted for the treatment of focal and paroxysmal atrial fibrillation, the anatomy of the pulmonary vein is still not fully understood. To investigate the dimensions and anatomical variation of the pulmonary vein in patients with paroxysmal atrial fibrillation, we performed breath-hold gadolinium enhanced magnetic resonance (MR) angiography using a 1.5 T cardiac MR imager (GE CV/i) in 32 patients with paroxysmal atrial fibrillation (61 +/- 8 years old), 11 patients with chronic atrial fibrillation (64 +/- 9 years old), and 26 patients with normal sinus rhythm (55 +/- 15 years old). Three dimensional images of the pulmonary veins were thus obtained, and the diameters of the most proximal portion of the left or right superior pulmonary vein and left or right inferior pulmonary vein were measured. Pulmonary vein branching variations were determined by a visual qualitative analysis by two separate readers' agreements, who were blinded to any clinical information. We focused on the existence of a complex-branching pattern draining into the orifice of four pulmonary veins. Patients with either paroxysmal atrial fibrillation or chronic atrial fibrillation showed larger superior pulmonary veins than those with normal sinus rhythm (mean +/- SD; in the left superior pulmonary vein, 20 +/- 3 mm, 23 +/- 3 mm vs 16 +/- 3 mm, P < 0.05; in right superior pulmonary vein, 19 +/- 4 mm, 19 +/- 2 mm vs 16 +/- 2 mm, P < 0.05). Complex-branching pattern was frequently observed in inferior pulmonary veins in patients with either paroxysmal atrial fibrillation or chronic atrial fibrillation; 25/32 patients with paroxysmal atrial fibrillation, 11/11 patients with chronic atrial fibrillation, compared to 7/26 patients with normal sinus rhythm. Complex-branching patterns were not observed in superior pulmonary veins in any patients in this cohort. In patients with paroxysmal atrial fibrillation or chronic atrial fibrillation, significant pulmonary vein dilation occurred mainly in the superior pulmonary veins, while a complex-branching pattern was frequently observed in the inferior pulmonary veins. These MR angiographic findings might be useful when performing radiofrequency ablation procedures and catheter manipulation for the treatment of paroxysmal atrial fibrillation.

NEUREGULIN-1 INDUCES BRANCHING MORPHOGENESIS IN THE DEVELOPING LUNG THROUGH A PI3K SIGNAL PATHWAY

Neuregulin-1 (NRG-1) induces signal transduction through the activation of its receptor, a heterodimer of human epidermal growth factor receptors 2 and 3 (HER2/HER3). Signal transduction through this receptor/ligand system plays a critical role in the developing heart, mammary gland, and nervous systems. Previous studies showed that NRG-1–induced HER2 activation resulted in pulmonary epithelial cell proliferation in the human fetal lung. The authors hypothesized that NRG-1 further contributes to lung development and maturation by inducing branching morphogenesis. In the present study, the authors show that NRG-1, HER2, and HER3, but not HER4, are expressed in the developing mouse lung. Addition of NRG-1 to fetal lung explants increased lung branching morphogenesis by 32% (P < .05). This increase in branching was blocked by 2C4, an antibody directed against HER2 that inhibits its dimerization and subsequent NRG-1–induced signal transduction. To gain an understanding of the intracellular signaling pathways involved in NRG-1–induced branching morphogenesis, the authors specifically blocked the phosphatidylinositol-3 kinase (PI3K) and mitogen activation protein kinase (MAPK) pathways. Inhibition of PI3K signaling significantly decreased NRG-1–induced branching morphogenesis (P < .05). Inhibition of NRG-1–induced MAPK activation had no effect on explant branching morphogenesis. These data suggest that NRG-1, binding to the HER2/HER3 heterodimer receptor complex, induces pulmonary branching morphogenesis through HER2 activation of the PI3K pathway.

Upregulated expression of EGF and TGF-? in the proximal respiratory epithelium in the human hypoplastic lung in congenital diaphragmatic hernia

Newborn infants with congenital diaphragmatic hernia (CDH) still have a high mortality rate. Epidermal growth factor (EGF) and transforming growth factor-alpha (TGF-alpha) are peptide growth factors involved in the fetal lung growth and development. The EGF and TGF-alpha have been reported to promote pulmonary branching activity and alveolar type-II pneumocyte proliferation. Epidermal growth factor and TGF-alpha immunoreactivity and mRNA expression in the bronchial and bronchiolar epithelium is maximal during early fetal life and barely detectable in the proximal airways of neonatal lung. The purpose of this study was to determine protein and gene expression of EGF and TGF-alpha in CDH lung in order to elucidate the potential role of these growth factors in the pathogenesis of pulmonary hypoplasia in CDH. Lung tissue specimens were obtained from archival lung tissue from 11 patients with CDH and 5 controls. Indirect immunohistochemistry was performed using ABC method with anti-EGF and anti-TGF-alpha antibodies. In situ hybridization was performed using EGF and TGF-alpha specific digoxigenin-labeled oligonucleotide probes. The most striking difference between hypoplastic CDH lung and control lung was the strong EGF and TGF-alpha mRNA expression and immunoreactivity in the bronchial and bronchiolar epithelium in CDH lung. The upregulated protein and gene expression of EGF and TGF-alpha in the proximal airways in the CDH hypoplastic lung suggests persistence of fetal stage of pulmonary airway development in CDH.

Terminology of three-dimensional and four-dimensional ultrasound imaging of the fetal heart and other moving body parts.

Terminology of three-dimensional and four-dimensional ultrasound imaging of the fetal heart and other moving body parts.

Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta.

The aim was to review our experience with the surgical repair of the anomalous origin of one pulmonary branch from the aorta (AOPA). Between January 1991 and March 2002, eight patients with AOPA underwent surgical correction. Three patients presented isolated AOPA. Five patients presented right AOPA and three, left AOPA. Implantation of the AOPA to the main pulmonary artery was performed by: (I) direct anastomosis in two patients with left AOPA; (II) interposition of a synthetic graft in one patient with left AOPA; (III) employing an autologous pericardial patch in two patients with right AOPA; (IV) using an aortic flap in three other patients with right AOPA. The mean follow-up time was 37.7 months. One patient died postoperatively due to progressive heart failure unresponsive to inotropic support. Early postoperative pulmonary hypertension crisis was identified in another patient. Within 1 year after surgery, the mean residual gradient across the anastomotic site at follow-up was 14+/-8 mmHg. The patient undergoing interposition of a synthetic graft presented a residual gradient of 29 mmHg and underwent reoperation at almost 2.5 years after the first correction. The residual gradient in patients undergoing correction according to technique I was 17+/-3 mmHg, and in patients undergoing implantation of the AOPA according to techniques III or IV was 9.5+/-4.6 mmHg (P=0.11). Similarly, the Tc-99m scintigraphy demonstrated that a lower lung perfusion (the lung perfused from the respective AOPA compared with the contralateral lung) in patients undergoing AOPA implantation according to technique I was 59+/-6(%) and in patients undergoing techniques III or IV was 72+/-4.5(%) (P=0.038). At follow-up, all patients were alive. The AOPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients presenting this anomaly may undergo correction using various surgical techniques with acceptable results. The techniques employing autologous tissues for enlarging and lengthening the AOPA seems to be associated with less restenosis at the anastomotic site, however, larger series of patients are required to confirm such outcome.

Invited commentary

This report by Watanabe and colleagues introduces an interesting technique that may be of significant value in planning lung resections. Obviously, one must question the cost of CT angiography in all routine lung resections. The authors imply that since routine CT scanning is a standard method in the preoperative evaluation of patients with known or suspected lung cancer, there would be no additional cost for processing data to provide 3D-CTA. However, the data processing requires increased technician time, and evaluation by a radiologist who will certainly bill. It would be hard to justify the use of this technique for the routine open thoracotomy lobectomy for a peripheral cancer. The authors correctly point out the great degree of variability in the branching of pulmonary arteries. However, since most experienced thoracic surgeons are well aware of the potential abnormalities, and have developed dissection techniques to search for them, the risk of inadvertently injuring an unsuspected abnormal vessel is quite low. There are certain circumstances where precise preoperative knowledge of the anatomy would be of great help. One example is in the operative planning for a patient with limited pulmonary reserve and a central tumor questionably involving the main pulmonary artery, for instance at the origin of the apical anterior branch. Involvement with tumor presents a high risk for requiring a pneumonectomy. Foreknowledge of the anatomy in such a patient might prevent needless exploration. The authors note the ability to perform video-assisted lobectomy, a technique that is becoming routinely performed by many surgeons. Such lobectomies are frequently performed in a sequence different from standard lobectomies, and the ability to carefully dissect pulmonary branches would be greatly enhanced by a road map of the patient’s anatomy. We have used this technique in selecting donors for living related lower lobe donations to recipients with cystic fibrosis. In these operations, the branching of pulmonary arteries is critical. When problems occur, they are usually on the left side where the relationship between the upper lobe lingular branch and the lower lobe superior segmental branch is critical. We have had the misfortune of encountering unfavorable situations such as a great distance between these two branches, or multiple superior segment branches. In such circumstances, use of the lower lobe requires a difficult implantation, sacrificing the donor lingular artery or performing a complex graft to revascularize this vessel. Thus, when we have two or three potential donors, we perform CT angiography to select appropriate donors and sides.

Semaphorin E and its receptors neuropilin 1 & 2 in human endometrium

Objective: Semaphorin E (SemaE), a class 3 semaphorin, guides axonal projections in embryonic brain development through receptors Neuropilin (NRP) 1 & 2. SemaE has also been demonstrated in non-neuronal tissue directing fetal pulmonary alveolar branching. Neuropilins also participate in angiogenesis by binding and mediating VEGF signaling. Previously, we reported differential expression of SemaE in human endometrium at different stages of the menstrual cycle by microarray analysis. Since angiogenesis and glandular differentiation represent major physiological changes in human endometrium, we postulate a potential signaling role for SemaE in the endometrium. Herein, we investigated the expression and hormonal regulation of SemaE, NRP 1 & 2 in the human endometrium.

Prenatal prediction of pulmonary hypoplasia: clinical, biometric, and Doppler velocity correlates.

To determine the value of pulmonary artery Doppler velocimetry relative to fetal biometric indices and clinical correlates in the prenatal prediction of lethal lung hypoplasia (LH) in prolonged (>1 week) oligohydramnios. Forty-two singleton pregnancies with oligohydramnios associated with premature rupture of membranes ([PROM]; n = 31) or bilateral renal pathology (n = 11) were examined using color-coded Doppler ultrasound in a cross-sectional study design. Mean gestational age was 28.0 +/- 4.3 weeks (range: 20-36 weeks). Thoracic, cardiac, and abdominal circumference and the largest vertical amniotic fluid pocket were measured. Pulsed Doppler measurements of the arterial pulmonary branches were made at the level of the cardiac 4-chamber view. Diagnosis of LH was based on clinical, radiologic, and pathologic criteria. Clinicians were blinded to the prenatal measurements. The prevalence of lethal LH was 43%. In the PROM subset, combination of onset of PROM at <or =20 weeks, duration of oligohydramnios at > or =8 weeks, and degree of oligohydramnios at < or =1 cm presented the highest clinical prediction rate for lethal LH. For both the total group and the PROM subset, the highest prediction rate for lethal LH was presented by thoracic circumference/abdominal circumference ratio, peak systolic velocity in the proximal branch, and time-averaged and end-diastolic velocity in the middle branch of the pulmonary artery. In the PROM subset, the combination of all 3 clinical, biometric, and Doppler parameters revealed the most favorable combination to predict lethal LH (positive predictive value: 100%; accuracy: 93%; and sensitivity: 71%). Doppler velocimetry may detect changes in pulmonary artery waveforms in the presence of LH but fails to be the ultimate test for the prenatal prediction of lethal LH. The best prediction can be achieved by combining clinical, biometric, and Doppler parameters.

Closure of the ductus arteriosus and development of pulmonary branch stenosis in babies of less than 32 weeks gestation

AIMSTo define how often transient pulmonary branch stenosis (PBS) develops after closure of a patent ductus arteriosus (PDA) in babies born at less than 32 weeks gestation; to describe the...