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1.
J Physiol ; 602(8): 1791-1813, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38532618

RESUMEN

Previous studies have suggested that an extended period of ventilation before delayed cord clamping (DCC) augments birth-related rises in pulmonary arterial (PA) blood flow. However, it is unknown whether this greater rise in PA flow is accompanied by increases in left ventricular (LV) output and systemic arterial perfusion or whether it reflects enhanced left-to-right shunting across the ductus arteriosus and/or foramen ovale (FO), with decreased systemic arterial perfusion. Using an established preterm lamb birth transition model, this study compared the effect of a short (∼40 s, n = 11), moderate (∼2 min, n = 11) or extended (∼5 min, n = 12) period of initial mechanical lung ventilation before DCC on flow probe-derived perinatal changes in PA flow, LV output, total systemic arterial blood flow, ductal shunting and FO shunting. The LV output was relatively stable during initial ventilation but increased after DCC, with similar responses in all groups. Systemic arterial flow patterns displayed only minor differences during brief and moderate periods of initial ventilation and were similar after DCC. However, an increase in PA flow was augmented with an extended initial ventilation (P < 0.001), owing to an earlier onset of left-to-right ductal and FO shunting (P < 0.001), and was accompanied by a pronounced reduction in total systemic arterial flow (P = 0.005) that persisted for 4 min after DCC (P ≤ 0.039). These findings suggest that, owing to increased left-to-right shunting and a greater reduction in systemic arterial perfusion, an extended period of ventilation before DCC does not result in greater perinatal circulatory benefits than shorter periods of initial ventilation in the birth transition. KEY POINTS: Previous studies suggest that an extended period of initial ventilation before delayed cord clamping (DCC) augments birth-related rises in pulmonary arterial (PA) blood flow. It is unknown whether this greater rise in PA flow is accompanied by an increased left ventricular output and systemic arterial perfusion or whether it reflects enhanced left-to-right shunting across the ductus arteriosus and/or foramen ovale, with decreased systemic arterial perfusion. Anaesthetized preterm fetal lambs instrumented with central arterial flow probes underwent a brief (∼40 s), moderate (∼2 min) or extended (∼5 min) period of ventilation before DCC. Perinatal changes in left ventricular output were similar in all groups, but extended initial ventilation augmented both perinatal increases in PA flow, owing to earlier onset and greater left-to-right ductal and foramen ovale shunting, and perinatal reductions in total systemic arterial perfusion. Extended ventilation before DCC does not confer a greater perinatal circulatory benefit than shorter periods of initial ventilation.


Asunto(s)
Conducto Arterial , Hipertensión Pulmonar , Embarazo , Femenino , Ovinos , Animales , Clampeo del Cordón Umbilical , Pulmón/irrigación sanguínea , Arteria Pulmonar/fisiología , Conducto Arterial/fisiología , Perfusión , Constricción
2.
Am J Med Genet A ; 194(3): e63458, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37921548

RESUMEN

Pathogenic variants in several genes involved in the function or regulation of smooth muscle cells (SMC) are known to predispose to congenital heart disease and thoracic aortic aneurysm and dissection (TAAD). Variants in MYLK are primarily known to predispose to TAAD, but a growing body of evidence points toward MYLK also playing an essential role in the regulation of SMC contraction outside the aorta. In this case report, we present a patient with co-occurrence of persistent ductus arteriosus (PDA) and thoracic aortic dissection. Genetic analyses revealed a novel splice acceptor variant (c.3986-1G > A) in MYLK, which segregated with disease in the family. RNA-analyses on fibroblasts showed that the variant induced skipping of exon 24, which resulted in an in-frame deletion of 101 amino acids. These findings suggest that MYLK-associated disease could include a broader phenotypic spectrum than isolated TAAD, including PDA and obstructive pulmonary disease. Genetic analyses could be considered in families with TAAD and PDA or obstructive pulmonary disease.


Asunto(s)
Aneurisma de la Aorta Torácica , Disección Aórtica , Azidas , Desoxiglucosa/análogos & derivados , Conducto Arterioso Permeable , Conducto Arterial , Enfermedades Pulmonares Obstructivas , Humanos , Masculino , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/metabolismo , Conducto Arterial/patología , Linaje , Disección Aórtica/genética , Conducto Arterioso Permeable/genética , Aneurisma de la Aorta Torácica/diagnóstico , Aneurisma de la Aorta Torácica/genética , Proteínas de Unión al Calcio/genética , Quinasa de Cadena Ligera de Miosina/genética , Quinasa de Cadena Ligera de Miosina/metabolismo
3.
Ultrasound Obstet Gynecol ; 63(5): 586-591, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38214544

RESUMEN

OBJECTIVE: Docosahexaenoic acid (DHA) is recommended routinely in pregnancy to promote fetal development. DHA has anti-inflammatory activity, but its effects on the fetal heart and circulation are unknown. This study aimed to investigate whether maternal DHA supplementation in the third trimester affects maternal prostaglandin levels and fetal ductus arteriosus flow dynamics. METHODS: This was a double-blind randomized controlled trial with parallel groups conducted between 2018 and 2021. Pregnant women aged over 18 years with a normal fetus at 27-28 weeks' gestation showing no cardiac/extracardiac anomalies or ductal constriction were eligible for the trial. Women consuming substances with a known inhibitory effect on prostaglandin metabolism, such as non-steroidal anti-inflammatory drugs and polyphenol-rich foods, were excluded. The intervention group received oral supplementation of omega-3 with 450 mg/day of DHA for 8 weeks and the placebo group received capsules of soy lecithin for 8 weeks. Anthropometric measurements, assessment of polyphenol and omega-3 consumption, fetal morphological ultrasound examination, fetal Doppler echocardiographic examination and blood sample collection were performed at the start of the study and the latter two were repeated at follow-up. Prostaglandin E2 (PGE2) level and echocardiographic parameters were compared between the intervention and placebo groups and between baseline and follow-up. RESULTS: A total of 24 participants were included in each group. After 8 weeks, there were no significant differences between the intervention and placebo groups in maternal serum PGE2 level or Doppler echocardiographic parameters of ductal flow. No case of ductus arteriosus constriction was observed. The expected intragroup changes in cardiac morphology, as a result of advancing gestation, were present. CONCLUSIONS: Maternal DHA supplementation in the third trimester at a clinically recommended dose did not result in inhibition of PGE2 or constriction of the ductus arteriosus. These findings should be confirmed in postmarket surveillance studies with larger patient numbers in order to test the full safety profile of DHA and provide robust clinical reassurance. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.


Asunto(s)
Suplementos Dietéticos , Ácidos Docosahexaenoicos , Conducto Arterial , Tercer Trimestre del Embarazo , Ultrasonografía Prenatal , Humanos , Femenino , Ácidos Docosahexaenoicos/administración & dosificación , Embarazo , Método Doble Ciego , Adulto , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/efectos de los fármacos , Conducto Arterial/embriología , Constricción Patológica
4.
Prenat Diagn ; 44(6-7): 899-906, 2024 06.
Artículo en Inglés | MEDLINE | ID: mdl-38797960

RESUMEN

OBJECTIVE: In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot (ToF) and its variations. This study aimed to determine whether there is an association of RAA and ADA in fetuses with ToF. Distribution of genetic anomalies and impact on postnatal outcome were further evaluated. METHOD: Single-center retrospective observational study including pregnancies with prenatal diagnosis of ToF from 2010 to 2023. All cases were subdivided into ToF with pulmonary stenosis (PS) and pulmonary atresia (PA). Clinical and echocardiographic databases were reviewed for pregnancy outcome, genetic anomalies, and postnatal course. RESULTS: The cohort included 169 cases, 124 (73.4%) with ToF/PS and 45(26.6%) with ToF/PA. Agenesis of the ductus arteriosus was significantly associated with RAA in both subtypes of ToF (p = 0.001) compared to left aortic arch and found in 82.5% (33/40) versus 10.7% (9/84) of fetuses with ToF/PS and in 57.1% (8/14) versus 12.9% (4/31) of fetuses with ToF/PA. In both ToF/PS and ToF/PA, RAA/ADA versus RAA/patent DA revealed a significantly higher risk for the presence of genetic abnormalities, especially microdeletion 22q11.2, major aorto-pulmonary collateral arteries and a shorter time to complete surgical repair. CONCLUSION: We demonstrated a significantly increased frequency of RAA/ADA in patients with prenatally diagnosed ToF. Although this association revealed no significant impact on overall survival, the prenatal detection of RAA/ADA has implications for counseling, genetic evaluation and postnatal management.


Asunto(s)
Aorta Torácica , Conducto Arterial , Tetralogía de Fallot , Ultrasonografía Prenatal , Humanos , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/epidemiología , Tetralogía de Fallot/genética , Femenino , Estudios Retrospectivos , Embarazo , Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Adulto , Conducto Arterial/anomalías , Conducto Arterial/diagnóstico por imagen , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/diagnóstico , Recién Nacido , Anomalías Múltiples/epidemiología , Anomalías Múltiples/diagnóstico por imagen
5.
Echocardiography ; 41(1): e15729, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38113302

RESUMEN

We described a case of a double aortic arch (DAA) with a subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC). We experienced uncertainty regarding this interesting case despite the diagnosis of right-sided ductus arteriosus. The ductus arteriosus originates from the right pulmonary artery (PA) and converges into the descending aorta (DAO), whereas the vessel originated from the PA and converged into the ascending aorta (AAO). Therefore, we assumed that the vessel connecting the PA to AAO may be a type-C persistent fifth aortic arch (PFAA).


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Anillo Vascular , Embarazo , Femenino , Humanos , Conducto Arterial/diagnóstico por imagen , Aorta Torácica/diagnóstico por imagen , Conducto Arterioso Permeable/diagnóstico por imagen , Aorta
6.
Acta Paediatr ; 113(5): 955-961, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38180109

RESUMEN

AIM: Transcatheter closure of the patent ductus arteriosus (TCPDA) is increasingly used in preterm infants as an alternative to surgical ligation. However, clinically ill preterm infants are at risk of contrast nephropathy due to the angiography contrast agents used during the procedure. METHODS: We performed a single-centre before-and-after comparative study in VLBW infants to compare the kinetics of serum creatinine during the first 4 days after TCPDA with or without angiography. RESULTS: 69 patients were included and divided into two groups: TCPDA with (contrast+; n = 37) and without (contrast-, n = 32) use of contrast agent. The median dose [range] of contrast agent was 1.0 mL/kg [0.6-2.4 mL/kg]. The change in serum creatinine level between day 2 to 4 after TCPCA and baseline decreased in the contrast- group (-17% [-46%; 18%]), while it increased in the contrast+ group (7% [-24%; 202%] p = 0.002). Comparison of blood urea levels between groups showed similar significant differences. The change in serum creatinine between day 2 to 4 and baseline was significantly correlated with the dose of contrast agent (r2 = 0.682; p < 0.001). CONCLUSION: The use of contrast agents during TCPDA can potentially harm the renal function of very preterm infants. Therefore, we advise minimising or avoiding the use of contrast agents.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Enfermedades del Prematuro , Lactante , Recién Nacido , Humanos , Recien Nacido Prematuro , Medios de Contraste/efectos adversos , Creatinina , Conducto Arterioso Permeable/diagnóstico por imagen , Riñón/diagnóstico por imagen , Resultado del Tratamiento
7.
J Ultrasound Med ; 43(3): 609-616, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38050801

RESUMEN

Advances in fetal echocardiography including newer techniques like 4D spatio-temporal image correlation technology has improved our understanding of fetal cardiac and extracardiac abnormalities. High resolution ultrasound combined with color Doppler and 3D rendering have contributed to an improved understanding of the fetal vascular system and its anomalies. This pictorial essay including ultrasound images and videos alongside their respective clay models, provides precise information of duct anatomy in fetuses with pulmonary atresia and aortic arch abnormalities.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Cardiopatías Congénitas , Atresia Pulmonar , Embarazo , Femenino , Humanos , Conducto Arterial/diagnóstico por imagen , Aorta Torácica/diagnóstico por imagen , Ultrasonografía Prenatal/métodos
8.
Pediatr Cardiol ; 45(3): 600-604, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38099950

RESUMEN

To evaluate the fetal ductus arteriosus anomalies diagnosed by fetal echocardiography. The perinatal outcomes and associated cardiac and genetic anomalies are also explored. The fetal echocardiography records of 2366 fetuses were evaluated retrospectively. Thirty-seven pregnancies prenatally diagnosed with ductus arteriosus anomalies and evaluated after delivery were enrolled in the study. Perinatal and obstetric outcomes were analyzed. The incidence of ductus arteriosus anomaly in our series was 1.5% (37/2366). The most frequent ductus arteriosus anomaly detected was right-sided ductus arteriosus followed by aneurysm, constriction and bilateral ductus arteriosus with an incidence of 51.3%, 27.1%, 18.9% and 2.7%, respectively. There were 19 fetuses with right-sided ductus arteriosus, of which 15 had tetralogy of Fallot. There were 2 chromosomal anomalies (22q11 microdeletion) in this group. Of the 7 fetuses with ductus arteriosus constriction, 3 (3/7, 42.9%) died in-utero. There were 2 (2/10, 20%) neonatal deaths due to hypoplastic left heart syndrome in the ductus arteriosus aneurysm group. Various types of ductus arteriosus anomalies can be diagnosed prenatally. Perinatal outcomes mostly dependent on the type of the ductus arteriosus anomaly and accompanying cardiac malformations.


Asunto(s)
Aneurisma , Conducto Arterioso Permeable , Conducto Arterial , Embarazo , Recién Nacido , Femenino , Humanos , Conducto Arterial/diagnóstico por imagen , Estudios Retrospectivos , Ultrasonografía Prenatal , Diagnóstico Prenatal , Conducto Arterioso Permeable/diagnóstico por imagen , Conducto Arterioso Permeable/epidemiología , Conducto Arterioso Permeable/complicaciones , Constricción Patológica , Aneurisma/complicaciones
9.
Pediatr Cardiol ; 45(4): 722-728, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38411710

RESUMEN

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.


Asunto(s)
Valvuloplastia con Balón , Conducto Arterioso Permeable , Conducto Arterial , Estenosis de la Válvula Pulmonar , Lactante , Femenino , Embarazo , Humanos , Recién Nacido , Estudios Retrospectivos , Ecocardiografía , Tercer Trimestre del Embarazo , Estenosis de la Válvula Pulmonar/diagnóstico por imagen , Estenosis de la Válvula Pulmonar/cirugía , Ultrasonografía Prenatal
10.
Cardiol Young ; 34(2): 314-318, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37403735

RESUMEN

The use of prostaglandin E1 is well documented in ductus arteriosus-dependent CHD or in neonatal pulmonary pathologies that cause severe pulmonary hypertension. The intravenous infusion is well established in loading infusion and maintenance with an onset of action of 30 minutes until 2 hours or even more. Our aim is to report three patients with pulmonary atresia that presented hypercyanotic spell due to a ductal spasm during cardiac catheterisation in whom the administration of a bolus of alprostadil reversed the spasm and increased pulmonary flow, immediately stabilising the condition of the patients allowing subsequent successful stent placement with no serious complications or sequelae after the administration of the bolus. More studies are needed to make a recommendation regarding the use of alprostadil in bolus in cases where the ductal spasm might jeopardise the life of the patient.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Cardiopatías Congénitas , Recién Nacido , Humanos , Alprostadil/uso terapéutico , Conducto Arterioso Permeable/tratamiento farmacológico , Espasmo
11.
Cardiol Young ; 34(2): 370-372, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37434447

RESUMEN

Type A3 truncus arteriosus describes pulmonary atresia with non-confluent mediastinal pulmonary arteries in which one pulmonary artery arises from a patent ductus arteriosus and the contralateral pulmonary artery from the aorta resulting in ductal dependent pulmonary blood flow. We describe a premature neonate with caudal regression syndrome and type A3 truncus arteriosus who was palliated with a ductal stent allowing completion of a prolonged neonatal ICU hospitalisation for multiple comorbidities.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Tronco Arterial Persistente , Recién Nacido , Humanos , Conducto Arterioso Permeable/complicaciones , Conducto Arterioso Permeable/cirugía , Tronco Arterial , Tronco Arterial Persistente/cirugía , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Stents
12.
Clin Anat ; 37(1): 140-146, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-37792451

RESUMEN

The ligamentum arteriosum (LA) is the vestigial fibrous remnant of the ductus arteriosus (DA), a fetal vessel arising from the left dorsal segment of the sixth aortic arch that connects the left pulmonary artery to the aortic arch. Incomplete obliteration of the DA results in a patent ductus arteriosus (PDA), causing the shunting of oxygen-rich blood to recirculate to the lungs, which can lead to pulmonary hypertension. The current study aims to further elucidate the structural characteristics of the LA via histological analysis with data gathered from adult cadaveric specimens. The LA was harvested and histologically observed with Hematoxylin and Eosin, van Gieson, and Masson's trichrome staining. Fibrous and muscle tissues were observed in all 25 specimens. The LA was categorized into three types based on the morphological features of the LA. Type I (vessel-like structure), type II (fibrotic tissue with duct-like structure), and type III (no duct-like structure) were found in 4.0%, 80.0%, and 16.0%, respectively. Finally, the remnant of a valve in the LA was also observed at the junction between the AA and LA. We suggest that this valve be called the "pulmonary-aortic valve." In the majority of the adult LAs, a duct-like structure was still present. These data could better elucidate our understanding of the pathology and etiology of a PDA.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Humanos , Adulto , Conducto Arterioso Permeable/patología , Aorta Torácica , Arteria Pulmonar , Conducto Arterial/patología , Aorta/patología
13.
J Clin Ultrasound ; 52(6): 737-744, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38662985

RESUMEN

OBJECTIVE: We aimed to analyze the echocardiographic characteristics and pregnancy outcomes for fetuses with premature complete closure of the fetal ductus arteriosus. METHODS: A retrospective analysis was performed for eight cases of premature ductus arteriosus closure diagnosed by prenatal ultrasonography in the Hunan Maternal and Child Health Hospital from July 2019 to August 2022, and the characteristics of fetal echocardiography and pregnancy outcomes of the eight cases were analyzed and summarized. RESULTS: In all cases, the intima of the ductus arteriosus was thickened and occluded, the ductus arteriosus could be seen with slightly hyperechogenic, and no blood flow signal was found in the ductus arteriosus by Doppler ultrasonography. The right heart was enlarged in seven cases, and the whole heart was enlarged in one case. Tricuspid valve regurgitation was observed to different degrees, of which seven cases were severe and one case was moderate. The pulmonary arteries of eight patients had varying degrees of widening. All eight cases were delivered by cesarean section, and one newborn died after follow-up. The prognosis of the other newborns was good. CONCLUSION: The parameters of prenatal echocardiography are helpful for the prognosis of fetuses with premature closure of the ductus arteriosus. Early prenatal detection, close observation, and clinical guidance can be used to select the right time of delivery.


Asunto(s)
Conducto Arterial , Ecocardiografía , Resultado del Embarazo , Ultrasonografía Prenatal , Humanos , Femenino , Embarazo , Estudios Retrospectivos , Ultrasonografía Prenatal/métodos , Adulto , Conducto Arterial/diagnóstico por imagen , Ecocardiografía/métodos , Recién Nacido
14.
J Clin Ultrasound ; 52(6): 717-722, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38655706

RESUMEN

PURPOSE: To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly (EA). METHODS: A retrospective cohort study of 30 EA and 60 normal fetuses were enrolled. The EA group was divided into two groups: with RDAF (EA-RDAF group) and without RDAF (EA-NRDAF group). FAM was used to measure TAPSE of EA and normal fetuses, and Z-scores were calculated. The differences of FAM-TAPSE Z-score, gestational week (GW), maternal age (MA), and mitral valve-tricuspid valve distance (MTD) between three groups were compared. The correlation and binary logistic regression between FAM-TAPSE Z-score, GW, MA, MTD, and RDAF were analyzed. RESULTS: FAM-TAPSE Z-score was significantly lower in EA-RDAF group compared to other groups (p < 0.05). FAM-TAPSE Z-score, GW, and MA were negatively correlated with RDAF (p < 0.05), but no correlation was found between TR, MDT, and RDAF (p > 0.05). Multivariate logistic regression showed that FAM-TAPSE Z-score was an independent influencing factor for RDAF (OR = 0.102, p < 0.05). CONCLUSION: RV dysfunction is an independent factor leading to RDAF in EA fetus, which provides a feasible theoretical basis for further study on improvement of RV function through intrauterine treatment to delay and prevent the RDAF, to avoid death cycle and improve live-birth rate.


Asunto(s)
Anomalía de Ebstein , Válvula Tricúspide , Ultrasonografía Prenatal , Humanos , Anomalía de Ebstein/fisiopatología , Anomalía de Ebstein/diagnóstico por imagen , Femenino , Estudios Retrospectivos , Embarazo , Válvula Tricúspide/diagnóstico por imagen , Válvula Tricúspide/fisiopatología , Válvula Tricúspide/embriología , Ultrasonografía Prenatal/métodos , Adulto , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/fisiopatología , Función Ventricular Derecha/fisiología , Corazón Fetal/diagnóstico por imagen , Corazón Fetal/fisiopatología , Estudios de Cohortes , Sístole , Ecocardiografía/métodos
15.
Kyobu Geka ; 77(6): 442-445, 2024 Jun.
Artículo en Japonés | MEDLINE | ID: mdl-39009538

RESUMEN

We present the case of 60s male who underwent ductus arteriosus closure at the age of 10. He presented with hoarseness and a 25 mm-sized saccular aortic aneurysm was identified at the site of the closed ductus through the computed tomography( CT). The patient successfully underwent 1-debranch thoracic endovascular aortic repair resulting in improved hoarseness. While rare, several reports have documented aneurysm formation long after ductus arteriosus closure. Recent studies highlight favorable outcomes with endovascular repair. Despite its rarity, aneurysmal formation after ductus closure remains a serious complication. Given the increasing population of patients with prior ductus arteriosus closure and the discontinuation of long-term follow-up, awareness of the complication of aneurysmal formation is crucial. Not only congenital cardiologists but also general physicians should consider this differential diagnosis for patients presenting with symptoms such as hoarseness or back pain and a history of ductus closure.


Asunto(s)
Aneurisma de la Aorta Torácica , Procedimientos Endovasculares , Humanos , Masculino , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/diagnóstico por imagen , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/cirugía , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Conducto Arterioso Permeable/cirugía , Conducto Arterioso Permeable/diagnóstico por imagen , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Resultado del Tratamiento , Reparación Endovascular de Aneurismas
16.
Am J Physiol Heart Circ Physiol ; 325(4): H687-H701, 2023 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-37566109

RESUMEN

The ductus arteriosus (DA) is a vascular shunt that allows oxygenated blood to bypass the developing lungs in utero. Fetal DA patency requires vasodilatory signaling via the prostaglandin E2 (PGE2) receptor EP4. However, in humans and mice, disrupted PGE2-EP4 signaling in utero causes unexpected patency of the DA (PDA) after birth, suggesting another role for EP4 during development. We used EP4-knockout (KO) mice and acute versus chronic pharmacological approaches to investigate EP4 signaling in DA development and function. Expression analyses identified EP4 as the primary EP receptor in the DA from midgestation to term; inhibitor studies verified EP4 as the primary dilator during this period. Chronic antagonism recapitulated the EP4 KO phenotype and revealed a narrow developmental window when EP4 stimulation is required for postnatal DA closure. Myography studies indicate that despite reduced contractile properties, the EP4 KO DA maintains an intact oxygen response. In newborns, hyperoxia constricted the EP4 KO DA but survival was not improved, and permanent remodeling was disrupted. Vasomotion and increased nitric oxide (NO) sensitivity in the EP4 KO DA suggest incomplete DA development. Analysis of DA maturity markers confirmed a partially immature EP4 KO DA phenotype. Together, our data suggest that EP4 signaling in late gestation plays a key developmental role in establishing a functional term DA. When disrupted in EP4 KO mice, the postnatal DA exhibits signaling and contractile properties characteristic of an immature DA, including impairments in the first, muscular phase of DA closure, in addition to known abnormalities in the second permanent remodeling phase.NEW & NOTEWORTHY EP4 is the primary EP receptor in the ductus arteriosus (DA) and is critical during late gestation for its development and eventual closure. The "paradoxical" patent DA (PDA) phenotype of EP4-knockout mice arises from a combination of impaired contractile potential, altered signaling properties, and a failure to remodel associated with an underdeveloped immature vessel. These findings provide new mechanistic insights into women who receive NSAIDs to treat preterm labor, whose infants have unexplained PDA.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Ratones , Animales , Recién Nacido , Femenino , Embarazo , Humanos , Conducto Arterial/metabolismo , Dinoprostona/metabolismo , Subtipo EP4 de Receptores de Prostaglandina E/genética , Subtipo EP4 de Receptores de Prostaglandina E/metabolismo , Conducto Arterioso Permeable/genética , Ratones Noqueados
17.
Arterioscler Thromb Vasc Biol ; 42(6): 732-742, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35443793

RESUMEN

OBJECTIVE: Failure to close the ductus arteriosus, patent ductus arteriosus, accounts for 10% of all congenital heart defects. Despite significant advances in patent ductus arteriosus management, including pharmacological treatment targeting the prostaglandin pathway, a proportion of patients fail to respond and must undergo surgical intervention. Thus, further refinement of the cellular and molecular mechanisms that govern vascular remodeling of this vessel is required. METHODS: We performed single-cell RNA-sequencing of the ductus arteriosus in mouse embryos at E18.5 (embryonic day 18.5), and P0.5 (postnatal day 0.5), and P5 to identify transcriptional alterations that might be associated with remodeling. We further confirmed our findings using transgenic mouse models coupled with immunohistochemistry analysis. RESULTS: The intermediate filament vimentin emerged as a candidate that might contribute to closure of the ductus arteriosus. Indeed, mice with genetic deletion of vimentin fail to complete vascular remodeling of the ductus arteriosus. To seek mechanisms, we turned to the RNA-sequencing data that indicated changes in Jagged1 with similar profile to vimentin and pointed to potential links with Notch. In fact, Notch3 signaling was impaired in vimentin null mice and vimentin null mice phenocopies patent ductus arteriosus in Jagged1 endothelial and smooth muscle deleted mice. CONCLUSIONS: Through single-cell RNA-sequencing and by tracking closure of the ductus arteriosus in mice, we uncovered the unexpected contribution of vimentin in driving complete closure of the ductus arteriosus through a mechanism that includes deregulation of the Notch signaling pathway.


Asunto(s)
Conducto Arterioso Permeable , Conducto Arterial , Animales , Conducto Arterial/metabolismo , Conducto Arterioso Permeable/genética , Conducto Arterioso Permeable/metabolismo , Humanos , Filamentos Intermedios/metabolismo , Ratones , ARN , Remodelación Vascular , Vimentina/genética , Vimentina/metabolismo
18.
Ultrasound Obstet Gynecol ; 61(3): 346-355, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36565437

RESUMEN

OBJECTIVES: Hemodynamic abnormalities and brain development disorders have been reported previously in fetuses and infants with transposition of the great arteries and intact ventricular septum (TGA-IVS). A ventricular septal defect (VSD) is thought to be an additional risk factor for adverse neurodevelopment, but literature describing this population is sparse. The objectives of this study were to assess fetal cardiac hemodynamics throughout pregnancy, to monitor cerebral hemodynamics and oxygen metabolism in neonates, and to compare these data between patients with TGA-IVS, those with TGA-VSD and age-matched controls. METHODS: Cardiac hemodynamics were assessed in TGA-IVS and TGA-VSD fetuses and compared with healthy controls matched for gestational age (GA) during three periods: ≤ 22 + 5 weeks (GA1), 27 + 0 to 32 + 5 weeks (GA2) and ≥ 34 + 5 weeks (GA3). Left (LVO), right (RVO) and combined (CVO) ventricular outputs, ductus arteriosus flow (DAF, sum of ante- and retrograde flow in systole and diastole), diastolic DAF, transpulmonary flow (TPF) and foramen ovale diameter were measured. Aortic (AoF) and main pulmonary artery (MPAF) flows were derived as a percentage of CVO. Fetal middle cerebral artery and umbilical artery (UA) pulsatility indices (PI) were measured and the cerebroplacental ratio (CPR) was derived. Bedside optical brain monitoring was used to measure cerebral hemoglobin oxygen saturation (SO2 ) and an index of microvascular cerebral blood flow (CBFi ), along with peripheral arterial oxygen saturation (SpO2 ), in TGA-IVS and TGA-VSD neonates. Using hemoglobin (Hb) concentration measurements, these parameters were used to derive cerebral oxygen delivery and extraction fraction (OEF), as well as an index of cerebral oxygen metabolism (CMRO2i ). These data were acquired in the early preoperative period (within 3 days after birth and following balloon atrial septostomy) and compared with those of age-matched healthy controls, and repeat measurements were collected before discharge when vital signs were stable. RESULTS: LVO was increased in both TGA groups compared with controls throughout pregnancy. Compared with controls, TPF was increased and diastolic DAF was decreased in TGA-IVS fetuses throughout pregnancy, but only during GA1 and GA2 in TGA-VSD fetuses. Compared with controls, DAF was decreased in TGA-IVS fetuses throughout pregnancy and in TGA-VSD fetuses at GA2 and GA3. At GA2, AoF was higher in TGA-IVS and TGA-VSD fetuses than in controls, while MPAF was lower. At GA3, RVO and CVO were higher in the TGA-IVS group than in the TGA-VSD group. In addition, UA-PI was lower at GA2 and CPR higher at GA3 in TGA-VSD fetuses compared with TGA-IVS fetuses. Within 3 days after birth, SpO2 and SO2 were lower in both TGA groups than in controls, while Hb, cerebral OEF and CMRO2i were higher. Preoperative SpO2 was also lower in TGA-VSD neonates than in those with TGA-IVS. From preoperative to predischarge periods, SpO2 and OEF increased in both TGA groups, but CBFi and CMRO2i increased only in the TGA-VSD group. During the predischarge period, SO2 was higher in TGA-IVS than in TGA-VSD neonates, while CBFi was lower. CONCLUSIONS: Fetal cardiac and neonatal cerebral hemodynamic/metabolic differences were observed in both TGA groups compared with controls. Compared to those with TGA-IVS, fetuses with TGA-VSD had lower RVO and CVO in late gestation. A higher level of preoperative hypoxemia was observed in the TGA-VSD group. Postsurgical cerebral adaptive mechanisms probably differ between TGA groups. Patients with TGA-VSD have a specific physiology that warrants further study to improve neonatal care and neurodevelopmental outcome. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.


Asunto(s)
Conducto Arterial , Defectos del Tabique Interventricular , Transposición de los Grandes Vasos , Lactante , Recién Nacido , Femenino , Humanos , Embarazo , Defectos del Tabique Interventricular/cirugía , Hemodinámica/fisiología , Arteria Pulmonar , Oxígeno , Hemoglobinas
19.
Pediatr Dev Pathol ; 26(4): 388-393, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37249143

RESUMEN

BACKGROUND: Differences in the shape of the ductus arteriosus (DA), an important vascular shunt between the pulmonary artery and aorta, may reflect fetoplacental blood flow. Our aim was to examine tapering of the DA in a fetal autopsy population and correlate it with placental pathology and cause of death (COD). METHODS: This autopsy case control study of stillborn fetuses selected cases (tapered DA) and consecutive age-matched controls (no DA tapering) between January 2017 and January 2022. We abstracted demographic and clinical data from pathology reports. Autopsy data included COD and histologic evidence of fetal hypoxia. Placental pathology included umbilical cord abnormalities, acute and chronic inflammation, fetal vascular malperfusion (FVM), and maternal vascular malperfusion (MVM). RESULTS: We identified 50 cases and 50 controls. Gestational age ranged from 18 to 38 weeks. Maternal and fetal demographic characteristics did not differ significantly between cases and controls. COD related to an umbilical cord accident/FVM was significantly more prevalent in cases vs controls (46% vs 26%, P = .037), and FVM in the placenta, regardless of COD, trended higher in cases than controls. CONCLUSION: Tapering of the DA is present in stillborn fetuses and associated with COD related to fetal vascular blood flow obstruction.


Asunto(s)
Conducto Arterial , Enfermedades Placentarias , Enfermedades Vasculares , Embarazo , Femenino , Humanos , Lactante , Placenta/patología , Autopsia , Estudios de Casos y Controles , Conducto Arterial/patología , Causas de Muerte , Mortinato , Enfermedades Placentarias/patología , Enfermedades Vasculares/patología
20.
Echocardiography ; 40(3): 295-298, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36789919

RESUMEN

Double aortic arch (DAA) with subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus (RDA) was not reported before delivery, only in adults with anatomy course findings. We present a case of fetal DAA with subaortic LBCV and RDA using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC).


Asunto(s)
Conducto Arterial , Anillo Vascular , Embarazo , Femenino , Adulto , Humanos , Conducto Arterial/diagnóstico por imagen , Aorta Torácica/diagnóstico por imagen , Venas Braquiocefálicas/diagnóstico por imagen , Ultrasonografía Prenatal/métodos , Diagnóstico Prenatal/métodos
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