Echocardiography in the diagnosis of Shone’s complex and analysis of the causes for missed diagnosis and misdiagnosis

BACKGROUND Shone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients.AIM To use echocardiography in the diagnosis of Shone’s complex and analyze the causes of missed diagnosis and misdiagnosis.METHODS This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14,2008,to November 22,2019.The patients were followed once a year at the outpatient clinic after surgery.RESULTS Sixty-six patients were included.The patients were 2.7(0.8-5.6)years of age,and 54.5%were male.Ten(15.2%)had a history of heart surgery.The most common heart defect was the Annulo-Leaflet mitral ring(ALMR)(50/66,75.8%),followed by coarctation of the aorta(CoA)(43/66,65.2%).The patients had a variety of combinations of defects.Only two(3.0%)patients had all four defects.None of the patients had a family history of congenital heart disease.The preoperative echocardiographic findings were examined against the intraoperative findings.Echocardiography missed an ALMR in 31 patients(47.0%),a parachute mitral valve(PMV)in one patient(1.5%),subaortic stenosis in one patient(1.5%),and CoA in two patients(3.0%).CONCLUSION Echocardiography is an effective method to diagnose the Shone’s complex.Due to this disease’s complexity and interindividual variability, Improving the understanding of thedisease can reduce misdiagnosis and missed diagnosis.

Two approaches for newborns with critical congenital heart disease: a comparative study

Background Prenatal diagnosis and planned peripartum care is an unexplored concept in China.This study aimed to evaluate the effects of the“prenatal diagnosis and postnatal treatment integrated model”for newborns with critical congenital heart disease.Methods The medical records of neonates(≤28 days)admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020.The patients were divided into“prenatal diagnosis and postnatal treatment integrated group”(n=47)and“non-integrated group”(n=69).Results The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group(5.2±7.2 days vs.11.8±8.0 days,P<0.001;11.9±7.0 days vs.16.5±7.7 days,P=0.001,respectively).The weight at surgery also was lower in the integrated group than in the non-integrated group(3.3±0.4 kg vs.3.6±0.6 kg,P=0.010).Longer postoperative recovery time was needed in the integrated group,with a median mechanical ventilation time of 97 h(interquartile range 51–259 h)vs.69 h(29–168 h)(P=0.030)and with intensive care unit time of 13.0 days(8.0–21.0 days)vs.9.0 days(4.5–16.0 days)(P=0.048).No significant difference was observed in the all-cause mortality(2.1 vs.8.7%,P=0.238),but it was significantly lower in the integrated group for transposition of the great arteries(0 vs.18.8%,log rank P=0.032).Conclusions The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns,and surgical intervention could be performed with a lower risk of death,especially for transposition of the great arteries.

Handmade tri-leaflet ePTFE conduits versus homografts for right ventricular outflow tract reconstruction

Background This study aimed to investigate the performance of handmade tri-leaflet expanded polytetrafluoroethylene(ePTFE)conduits in the absence of a suitable homograft.Methods Patients who underwent right ventricular outflow tract reconstruction with tri-leaflet ePTFE conduits or homografts between December 2016 and August 2020 were included.The primary endpoint was the incidence of moderate or severe conduit stenosis(>36 mmHg)and/or moderate or severe insufficiency.The secondary endpoint was the incidence of severe conduit stenosis(>64 mmHg)and/or severe insufficiency.Results There were 102 patients in the ePTFE group and 52 patients in the homograft group.The median age was younger[34.5(interquartile range:20.8-62.8)vs.60.0(interquartile range:39.3-81.0)months,P=0.001]and the median weight was lower[13.5(10.0-19.0)vs.17.8(13.6-25.8)kg,P=0.003]in the ePTFE group.The conduit size was smaller(17.9±2.2 vs.20.5±3.0 mm,P<0.001)and the conduit Z score was lower(1.48±1.04 vs.1.83±1.05,P—0.048)in the ePTFE group.There was no significant difference in the primary endpoints(log rank,T3=0.33)and secondary endpoints(log rank,P=0.35).Multivariate analysis identified lower weight at surgery[P=0.01;hazard ratio:0.75;95%confidence interval(CI)0.59-0.94]and homograft conduit use(P=0.04;hazard ratio:8.43;95%CI 1.14—62.29)to be risk factors for moderate or severe conduit insufficiency.No risk factors were found for moderate or severe conduit stenosis or conduit dysfunction on multivariate analysis.Conclusion Handmade tri-leaflet ePTFE conduits showed acceptable early and midterm outcomes in the absence of a suitable homograft,but a longer follow-up is needed.