Bronchoscopy in the management of children from developing countries undergoing congenital heart surgery.

OBJECTIVES: To study the clinical characteristics and impact of bronchoscopy in children from developing countries, referred for cardiac surgery, through the "Save a Child's Heart" (SACH) organization. METHODS: We performed a retrospective hospital-chart review of SACH children (0-18 years old) referred between 2006 and 2021 who underwent fiberoptic bronchoscopy. We examined demographics, congenital-heart-disease (CHD) types, bronchoscopy's indications and findings, subsequent recommendations, number of ventilation, and intensive-care-unit days. The primary outcome was percent changes in management and diagnosis, following the bronchoscopy. We included a control group matched-for-age and CHD type, who did not undergo bronchoscopy. RESULTS: We performed 82 bronchoscopies in 68 children: 18 (26.5%) preoperatively; 46 (67.6%) postoperatively; and four (5.9%) both. The most prevalent CHDs were Tetralogy-of-Fallot (27.9%) and ventricular-septal-defect (19.1%). The main indications were persistent atelectasis (41%) and mechanical ventilation/weaning difficulties (27.9%). Bronchoscopic evaluations revealed at least one abnormality in 51/68 (75%) children. The most common findings were external airway compression (23.5%), bronchomalacia (19.1%), and mucus secretions (14.7%). Changes in management were made in 35 (51.4%) cases, with a major change made in 14/35 (40%) children. Compared to the control group, the children undergoing bronchoscopy were both ventilated longer (median 6 vs. 1.5 days, p < 0.0001) and stayed longer in the intensive care unit (median 1.5 vs. 18.5 days, p < 0.0001). CONCLUSION: A bronchoscopy is an important tool in the diagnosis and management of the unique group of children from developing countries with CHD referred for cardiac surgery. The results of our study, reveal a more complicated clinical course in children requiring bronchoscopy compared to controls.

Isolated ventricular septal defects demonstrated by fetal echocardiography: prenatal course and postnatal outcome.

OBJECTIVES: We assessed the natural history of the different types of isolated ventricular septal defects (VSDs) diagnosed by fetal echocardiography and analyzed their postnatal outcomes. METHODS: This is a retrospective cohort study of 86 fetuses with isolated VSDs, detected in 7466 sequential echocardiographic examinations. The subtype and size of the VSDs were assessed during fetal life and the following birth. Data on the spontaneous closure of the VSD, need for intervention, additional abnormalities and chromosomal aberrations was analyzed. RESULTS: From the original cohort 75 cases of isolated VSDs with complete data on outcome were further analyzed. Muscular and perimembranous VSDs were found in 85.3 and 14.7%, respectively. Spontaneous closure of the VSDs occurred prenatally in 31/64 and 3/11 of fetuses with muscular VSD and perimembranous VSD, respectively. Spontaneous closure of the VSD by the age of 2 years occurred in 92.2 and 45.5% of cases with muscular and perimembranous VSDs respectively (p = 0.001). CONCLUSION: Isolated muscular VSDs usually close spontaneously during pregnancy or in the first 2 years of life and probably do not increase the risk for chromosomal aberrations. On the other hand, isolated perimembranous VSDs may need intervention following birth and may be associated with a chromosomal anomaly.

Can Fetal Echocardiographic Measurements of the Left Ventricular Outflow Tract Angle Detect Fetuses with Conotruncal Cardiac Anomalies?

OBJECTIVES: The angle between the inter-ventricular septum and the ascending aorta can be measured during a sonographic fetal survey while viewing the left ventricular outflow tract (LVOT angle). Our aim was to compare the LVOT angle between fetuses with and without conotruncal cardiac anomaliesrmations. METHODS: In this prospective observational study, we compared the LVOT angle between normal fetuses, at different gestational age, and fetuses with cardiac malformations. RESULTS: The study included 302 fetuses screened at gestational age of 12-39 weeks. The LVOT angle ranged from 127 to 163 degrees (mean 148.2), in 293 fetuses with normal hearts, and was not correlated with gestational age. The LVOT angle was significantly wider in fetuses with D-transposition of the great arteries (D-TGA, eight fetuses) and valvar aortic stenosis (AS, three fetuses), than in fetuses with normal hearts (164.8 ± 5.0 vs. 148.2 ± 5.4, respectively, p < 0.001). Conversely, the LVOT angle was significantly narrower in fetuses with complete atrioventricular canal defect (AVC, eight fetuses), than in fetuses with normal hearts (124.8 ± 2.4 vs. 148.2 ± 5.4, respectively, p < 0.001). On ROC analysis, an angle of 159.6 degrees or higher had a sensitivity of 100% and a specificity of 97.3% for the detection of TGA or AS, whereas an angle of 128.8 degrees or lower had a sensitivity of 100% and a specificity of 99.7% for the detection of AVC defect. CONCLUSIONS: The LVOT angle is constant during pregnancy, and differs significantly in fetuses with TGA/AS, and AVC, compared to fetuses with normal hearts (wider and narrower, respectively).

The association between severe fetal congenital heart defects and placental vascular malperfusion lesions.

OBJECTIVE: Studies have shown an association between infant with congenital heart defects (CHD) and the risk of preeclampsia. We aimed to characterize placental histopathology from pregnancies who underwent termination of pregnancy (TOP) because of severe CHD. METHODS: This was a case control study. The medical files of all TOPs due to fetal congenital malformations were reviewed. Cases with CHD included hypoplastic left heart, transposition of great arteries, AV canal, tetralogy of Fallot, double outlet RV, and coractation of aorta. The controls included TOPs due to congenital central nervous system defects (CNS group) that were matched in a 1:1 ratio, by gestational age and maternal age. Placental lesions were classified to maternal and fetal vascular malperfusion (MVM and FVM) and inflammatory lesions. RESULTS: Higher rates of any MVM or FVM lesion were observed in placentas from the CHD group (n = 32) as compared with the CNS group (n = 32), 40.6% versus 12.5% respectively, p = .02. As compared with the CNS group, the CHD group had more abnormal coiling of umbilical cord (p = .01). CONCLUSION: Placental vascular malperfusion lesions are more common in pregnancies complicated with CHD as compared with CNS malformations. These findings support the hypothesis of similar etiopathogenetic factors, contributing to the development of preeclampsia and CHD.

Bartonella quintana, an Unrecognized Cause of Infective Endocarditis in Children in Ethiopia.

Bartonella quintana endocarditis, a common cause of culture-negative endocarditis in adults, has rarely been reported in children. We describe 5 patients 7-16 years of age from Ethiopia with heart defects and endocarditis; 4 cases were caused by infection with B. quintana and 1 by Bartonella of undetermined species. All 5 patients were afebrile and oligosymptomatic, although 3 had heart failure. C-reactive protein was normal or slightly elevated, and erythrocyte sedimentation rate was high. The diagnosis was confirmed by echocardiographic demonstration of vegetations, the presence of high Bartonella IgG titers, and identification of B. quintana DNA in excised vegetations. Embolic events were diagnosed in 2 patients. Our data suggest that B. quintana is not an uncommon cause of native valve endocarditis in children in Ethiopia with heart defects and that possible B. quintana infection should be suspected and pursued among residents of and immigrants from East Africa, including Ethiopia, with culture-negative endocarditis.

Right ventricular outflow tract strategies for repair of tetralogy of Fallot: effect of monocusp valve reconstruction.

OBJECTIVES: The absence of a pulmonary valve (PV) after tetralogy of Fallot (TOF) repair has been shown to impact postoperative right ventricular (RV) function. The purposes of this study were to (i) compare early outcomes after PV-sparing vs transannular patching (TAP) with monocusp valve reconstruction or TAP alone and (b) assess the mid-term results after polytetrafluoroethylene (PTFE) membrane monocusp reconstruction. METHODS: From 2003 to 2009, 163 patients underwent TOF repair. Sixty-nine patients (42.3%) underwent a PV-sparing procedure (Group A), 74 (45.4%) underwent PTFE membrane monocusp valve reconstruction (Group B) and 20 (12.3%) underwent TAP only (Group C). Early outcomes were evaluated by the right-to-left ventricular pressure ratio, RV outflow tract gradient, tricuspid and PV function, intensive care unit (ICU) parameters and need for reintervention. Group B patients were also evaluated at intermediate term for clinical and echocardiographic parameters, including tricuspid and monocusp valve function and mobility. RESULTS: The median age, weight and PV Z-value of Group B patients were significantly lower; 20.5 months, 9.3 kg and -4, respectively. Postoperatively, the right-to-left ventricular pressure ratio was <0.5 in all groups. Mechanical ventilation time, fluid drainage duration and total ICU stay showed no significant difference between Groups A and B, while Group C was significantly longer (P < 0.01). There were five (3%) early deaths: three from Group A and two from Group B. The incidences of moderate or severe pulmonary insufficiency (PI) on discharge were 8.2% in Group A, 9% in Group B and 50% in Group C (P < 0.001). Among Group B patients, 85% of the evaluated patients had less than moderate PI in the intermediate-term follow-up, QRS duration <140 ms in 83.3% and right-to-left ventricular diameter ratio of 0.6 ± 0.2. Two (2.6%) patients underwent reoperation for monocusp replacement. There were two (2.7%) mid-term deaths. CONCLUSIONS: The use of a PTFE membrane monocusp valve and a valve-sparing strategy prevents immediate PI and improves short-term clinical outcomes. PTFE membrane monocusp appears advantageous in preventing severe intermediate-term PI and facilitates the preservation of RV function.

Coxiella burnetii infection of a bovine jugular vein conduit in a child.

We report a case of an 11-year-old girl with Coxiella burnetii infection of a bovine jugular vein conduit which is an extremely rare manifestation of Q fever. The role of surgery in the management of C. burnetii endovascular infection and the use of serology are discussed.