Christoph Wohlmuth

Tissue Doppler imaging in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome before and after fetal aortic valvuloplasty

Fetal aortic valvuloplasty can improve filling and reduce afterload of the left ventricle in critical aortic stenosis. Success of an intrauterine intervention is currently measured by technical success, clinical survival and eventual postnatal biventricular physiology. In the present study we investigated the use of tissue Doppler imaging (TDI) to evaluate changes in ventricular function assessed before and after prenatal aortic valvuloplasty.

Maternal aspects of fetal cardiac intervention.

Fetal cardiac interventions have the potential to alter natural disease progression and reduce morbidity and mortality in children. Although there are already encouraging data on fetal outcome, information on maternal morbidity and mortality after intervention is scarce. The aim of the present study was to assess maternal aspects, pregnancy‐associated risks and adverse events in 53 intrauterine cardiac interventions.

Fetal cardiovascular hemodynamics in twin–twin transfusion syndrome

Twin–twin transfusion syndrome (TTTS) complicates 10–15% of monochorionic–diamniotic (MCDA) pregnancies. It originates from unbalanced transfer of fluid and vasoactive mediators from one twin to its co‐twin via placental anastomoses. This results in hypovolemia in the donor and hypervolemia and vasoconstriction in the recipient twin. Consequently, the recipient demonstrates cardiovascular alterations including atrioventricular valve regurgitation, diastolic dysfunction, and pulmonary stenosis/atresia that do not necessarily correlate with Quintero‐stages. Selective fetoscopic laser photocoagulation of placental vascular anastomoses disrupts the underlying pathophysiology and usually improves cardiovascular function in the recipient with normalization of systolic and diastolic function within weeks after treatment. Postnatal studies have demonstrated early decreased arterial distensibility in ex‐donor twins, but 10‐year follow up is encouraging with survivors showing normal cardiovascular function after TTTS. However, prediction and appropriate early management of TTTS remain poor. Assessment of the cardiovascular system provides additional insight into the pathophysiology and severity of TTTS and may permit more targeted early surveillance of MCDA pregnancies in future. It should form an integral part of the diagnostic algorithm.

Granulomatous Mycosis Fungoides in an Adolescent-A Rare Encounter and Review of the Literature.

Granulomatous mycosis fungoides (GMF) is a rare form of mycosis fungoides (MF) characterized by an infiltrate of atypical lymphocytes, histiocytes, and multinucleated giant cells. Clinically, GMF has a slowly progressing course with a worse prognosis than other forms of MF. With its peak incidence being in the fifth to sixth decade, GMF is rare in children and adolescents. Herein we describe a 14‐year‐old boy with GMF.

Cardiac pathophysiology in twin–twin transfusion syndrome: new insights into its evolution

In twin–twin transfusion syndrome (TTTS), unbalanced transfer of vasoactive mediators and fluid from the donor to the recipient cotwin alters their cardiovascular function. The aims of this study were to describe the impact of TTTS on fetal cardiac function in a large cohort of monochorionic–diamniotic (MCDA) pregnancies, and determine the early hemodynamic response to selective fetoscopic laser photocoagulation (SFLP).

Aortic distensibility as a surrogate for intertwin pulse pressure differences in monochorionic pregnancies with and without twin-twin transfusion syndrome

Twin–twin transfusion syndrome (TTTS) complicates 10–15% of monochorionic diamniotic (MCDA) twin pregnancies. The donor response to hypovolemia allows the transfer of vasoactive mediators to the recipient, causing increased recipient afterload and hypertension. Our objective was to apply a novel speckle‐tracking technique to measure the aortic fractional area change (AFAC) during the cardiac cycle in MCDA twins with and without TTTS, and identify intertwin differences in AFAC and parameters of cardiac function.

Changes in ductus venosus flow profile in twin–twin transfusion syndrome: role in risk stratification

To evaluate changes in ductus venosus (DV) waveforms and the timing of these changes in twin–twin transfusion syndrome (TTTS), to relate these to disease severity and to assess the clinical applicability of the suggested measurements in the prediction of TTTS.

The biplane mode: it takes two to tango

The three vessels and trachea (3VT) view, the most cephalad of the five transverse planes in fetal echocardiography, is the gold standard for detection of arch anomalies1,2. Included is the double aortic arch, a relatively rare congenital heart defect characterized by an ascending aorta that bifurcates and forms a complete vascular ring encircling the trachea and esophagus. The hypothetical model of the aortic arch proposed by Edwards3 facilitates understanding normal and abnormal development; however, in practice, various branching patterns of the head and neck vessels (HNV) may be observed and will determine the postnatal surgical approach. While the 3VT view on its own is ideal for assessment of the arches and their relationships, it cannot visualize the HNV. Sagittal views on the other hand allow investigation of the HNV, but it is not possible to image both arches at the same time. We propose the use of the biplane mode to assess the relationship of both the HNV and the aortic arches. Using a GE Voluson E10 (GE Medical Systems, Zipf, Austria) ultrasound machine with an eM6C probe, high-resolution, high-frame rate images in two perpendicular planes can be displayed simultaneously. In the case of double aortic arch, the biplane mode combines the advantages of both acquisition planes and helps to identify the laterality of the HNV seen in the sagittal view by displaying simultaneously the 3VT view (Figure 1).

Recipient umbilical artery elongation (redundancy) in twin-twin transfusion syndrome

BACKGROUND: Chronic hypertension in adults causes arterial lengthening in major arteries, but the effects of early fetal hypertension on the twin‐twin transfusion syndrome recipients vascular architecture remains unknown. OBJECTIVE: We hypothesize that arterial cord redundancy is related to recipient hypertension and subsequent heart failure. Our objectives were to: (1) establish a 3‐dimensional color Doppler ultrasound method of measuring umbilical arterial length relative to its corresponding venous segment in the umbilical cord using artery vein angle; (2) compare recipient artery vein angle to gestational age‐matched controls; and (3) test the association of artery vein angle with recipient heart failure. STUDY DESIGN: We compared 3 groups prospectively: twin‐twin transfusion syndrome pregnancies undergoing fetoscopic laser surgery (preoperatively) and 2 groups of gestational age‐matched controls: uncomplicated monochorionic‐diamniotic twin pregnancies and healthy singletons. Using a 3‐dimensional color‐Doppler volume image of 5 cm of cord near the placental insertion, we traced the umbilical artery and vein producing umbilical artery:vein length, (artery vein index) and measured the artery vein angle between umbilical artery and vein. Correlation of artery vein angle to twin‐twin transfusion syndrome stage, maximum vertical pocket, umbilical arterial indices, ductus venosus Doppler, and brain natriuretic peptide were performed. We used pulsed‐wave and tissue Doppler to measure tissue Doppler velocities and indexed cardiac output and correlated these with artery vein angle. Comparative statistics, including multivariable linear regression, examined the relationship between umbilical arterial Doppler indices and artery vein angle. RESULTS: Artery vein angle and artery vein index correlated significantly (R2, 0.86; P < .0001), hence, artery vein angle was used for analysis. Mean artery vein angle was 33.1 ± 31.5 degrees in recipients (n = 44), 9.5 ± 6 degrees in monochorionic‐diamniotic (n = 11; 22 fetuses), and 8.9 ± 8.3 degrees in singleton controls (n = 16) (P < .001). An artery vein angle ≥26 degrees (>95th percentile for controls) was measured in 52% recipients. Artery vein angle was higher in twin‐twin transfusion syndrome stage 3R vs 1 (P = .001). Artery vein angle increased with increasing umbilical arterial pulsatility index (P < .001), and decreased with increasing resistance index (P = .02) after adjusting for gestational age. Interrater agreements to categorize abnormal artery vein angle values was 95% (P < .001). Abnormal ductus venosus Doppler and elevated recipient amniotic fluid N‐terminal pro‐brain natriuretic peptide/protein levels correlated significantly with artery vein angle. Abnormal artery vein angles were associated with decreased indexed cardiac output, lower tissue Doppler velocities, higher right‐sided Tei indices, and severe tricuspid regurgitation. CONCLUSION: Umbilical arterial lengthening occurs in 52% of recipients and is associated with abnormal Doppler flows, low systolic tissue Doppler velocities, reduced cardiac output, and elevated markers of cardiac failure. This may reflect chronicity and severity of hypertension in the recipient fetus. Further research is needed to explore the mechanisms of elongation and long‐term implications.

Ductus venosus alterations in twin‐twin transfusion syndrome: A useful tool for risk stratification

To evaluate changes in ductus venosus (DV) waveforms and the timing of these changes in twin–twin transfusion syndrome (TTTS), to relate these to disease severity and to assess the clinical applicability of the suggested measurements in the prediction of TTTS.