James Cnota

64 statements · 2 topics · summaries given as host listed separately

Featured statements

▶ Ep 2 · 14:35
For kids with a big VSD who don't have trisomy 13 or 18, we don't really worry about a fixed pulmonary vascular resistance problem until well into the first year of life. Uh, but this can happen much earlier in the first months, uh, in trisomy 13 and 18
quote · Trisomy 13
▶ Ep 2 · 29:30
if the heart is big and the lungs are flooded and the BMP is high, Then that's easy. We don't do a cath. We would entertain closing the hole.
quote · Trisomy 13
▶ Ep 2 · 17:55
the inpatient operative survival to discharge was over 90% for both trisomy 13 and 18.
quote · Trisomy 18
▶ Ep 2 · 2:10
Polyvalvular dysplasia (redundant and thickened valve) is a hallmark of trisomy 13-18, can affect any of the four cardiac valves, and typically is not an important driver of cardiac symptoms but points toward genetic diagnosis.
clinical · Trisomy 18

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James's statements about Trisomy 13 32 statements

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James Cnota, MD - 2024 Fetal Care Center Navigating Perinatal Care for Trisomy 13 & 18

▶ Ep 2 · 1:30
quote CHD is present in 80 to 90% of children. And all types are present, ranging from mild to moderate to severe in complexity. ↗
▶ Ep 2 · 1:30
epidemiological CHD is present in 80 to 90% of children with trisomy 13 and 18, with all types present ranging from mild to moderate to severe in complexity. ↗
▶ Ep 2 · 1:50
clinical The complexity seen in prenatal cohorts is typically more severe than in postnatal cohorts, which influences counseling and outcomes. ↗
▶ Ep 2 · 2:10
quote one of the hallmarks. Uh, of trisomy 13-18 is polyvacular dysplasia, which is a redundant and thickened, uh, valve. ↗
▶ Ep 2 · 2:10
clinical Polyvalvular dysplasia (redundant and thickened valve) is a hallmark of trisomy 13-18, can affect any of the four cardiac valves, and typically is not an important driver of cardiac symptoms but points toward genetic diagnosis. ↗
▶ Ep 2 · 4:40
epidemiological In trisomy 13, the most common lesions are patent ductus arteriosus (32%), atrial septal defects (27%), and ventricular septal defects (28%), all resulting in left-to-right shunting. ↗
▶ Ep 2 · 5:20
clinical Tetralogy of Fallot is the most common right-to-left shunt in trisomy 13, resulting in cyanosis depending on severity. ↗
▶ Ep 2 · 5:45
epidemiological In trisomy 18, ventricular septal defect is by far the most prevalent cardiac diagnosis. ↗
▶ Ep 2 · 8:40
clinical There is a very steep inflection in survival as gestational age increases for cardiac patients, with a particularly large gap between cardiac and non-cardiac patients in the 28 to 33 weeks gestation range. ↗
▶ Ep 2 · 10:00
clinical The airway is a challenging problem for cardiac surgery and can complicate liberating patients from mechanical ventilation after surgery for simple shunts. ↗
▶ Ep 2 · 10:25
clinical Pulmonary hypertension in trisomy 13/18 is unique in that it is early and accelerated in onset, which is guiding current trends in timing of cardiac surgery. ↗
▶ Ep 2 · 10:50
clinical Myocardial dysfunction seems to be more common in trisomy 13/18 patients and adds risk to cardiac interventions (institutional observation being investigated for publication). ↗
▶ Ep 2 · 14:10
clinical Some babies with trisomy 13/18 do not have the normal physiologic drop in pulmonary vascular resistance, which greatly increases surgical risk. ↗
▶ Ep 2 · 14:35
clinical For children with large VSD without trisomy 13/18, fixed pulmonary vascular resistance is not a concern until well into the first year of life, but in trisomy 13/18 this can happen much earlier in the first months. ↗
▶ Ep 2 · 14:35
quote For kids with a big VSD who don't have trisomy 13 or 18, we don't really worry about a fixed pulmonary vascular resistance problem until well into the first year of life. Uh, but this can happen much earlier in the first months, uh, in trisomy 13 and 18 ↗
▶ Ep 2 · 16:40
epidemiological Multi-center FIS database data show a clear trend of increasing numbers of cardiac surgeries being offered to trisomy 13/18 patients, with 74 centers involved. ↗
▶ Ep 2 · 17:30
epidemiological In the most contemporary multi-center dataset, only 12% of trisomy 13 and 17% of trisomy 18 patients underwent cardiac surgery. ↗
▶ Ep 2 · 17:55
epidemiological Inpatient operative survival to discharge was over 90% for both trisomy 13 and 18 in the multi-center study. ↗
▶ Ep 2 · 17:55
quote the inpatient operative survival to discharge was over 90% for both trisomy 13 and 18. ↗
▶ Ep 2 · 23:40
clinical Most PDAs in trisomy babies can be closed in the cath lab today, though some require surgical ligation based on ductal morphology. ↗
▶ Ep 2 · 26:00
clinical Ductal stenting has evolved in the last 10 years to be used widely for various forms of congenital heart disease with reduced pulmonary blood flow, including tetralogy of Fallot in trisomy patients. ↗
▶ Ep 2 · 26:30
clinical Pulmonary flow restrictors (vascular plugs with small holes) can be placed percutaneously in the cath lab to reduce left-to-right shunt symptoms as a palliative step, replacing historical use of pulmonary bands. ↗
▶ Ep 2 · 26:40
clinical Cincinnati Children's typically performs formal airway evaluation before cardiac surgery and screens for hepatoblastoma through referring centers. ↗
▶ Ep 2 · 27:00
epidemiological Since 2019, Cincinnati Children's has performed 17 index cardiopulmonary bypass procedures, mostly for simple shunts but including 6 cases of tetralogy and double outlet right ventricle, with 94% operative survival. ↗
▶ Ep 2 · 27:00
quote since 2019, we've done 17 um. Index, uh, uh, cardiopulmonary bypass, uh, procedures, um, mostly in simple shunts, but, uh, 6 of these were tetrology and double outlet right ventricle. And the operative survival at our center is right in the range of the Balt Center report at 94%. ↗
▶ Ep 2 · 28:00
opinion Improved outcomes are attributed to both careful case selection and institutional learning about postoperative intensive care for trisomy 13/18 patients. ↗
▶ Ep 2 · 28:00
quote I don't think it's all selection. I do think that selection is important, case selection. I mean, it's important for Every procedure. Right, uh, so case selection is important, but I also think that As you do more, you learn more. ↗
▶ Ep 2 · 28:30
clinical Assessment of pulmonary vascular bed status and airway status are critically important for case selection beyond just the cardiac disease. ↗
▶ Ep 2 · 29:30
quote if the heart is big and the lungs are flooded and the BMP is high, Then that's easy. We don't do a cath. We would entertain closing the hole. ↗
▶ Ep 2 · 29:30
clinical When the heart is enlarged, lungs are flooded, and BNP is high, cardiac catheterization is not needed before considering VSD closure. ↗
▶ Ep 2 · 29:55
clinical When a VSD is large and non-restrictive, there will never be a pressure gradient, so the RV will always be at systemic pressure. ↗
▶ Ep 2 · 30:15
clinical Cardiac catheterization is performed when the clinical puzzle doesn't fit together (small LV, baby not breathing fast) to assess pulmonary hypertension before surgery. ↗
James's statements about Trisomy 18 32 statements

Open the Trisomy 18 collection →

James Cnota, MD - 2024 Fetal Care Center Navigating Perinatal Care for Trisomy 13 & 18

▶ Ep 2 · 1:30
quote CHD is present in 80 to 90% of children. And all types are present, ranging from mild to moderate to severe in complexity. ↗
▶ Ep 2 · 1:30
epidemiological CHD is present in 80 to 90% of children with trisomy 13 and 18, with all types present ranging from mild to moderate to severe in complexity. ↗
▶ Ep 2 · 1:50
clinical The complexity seen in prenatal cohorts is typically more severe than in postnatal cohorts, which influences counseling and outcomes. ↗
▶ Ep 2 · 2:10
clinical Polyvalvular dysplasia (redundant and thickened valve) is a hallmark of trisomy 13-18, can affect any of the four cardiac valves, and typically is not an important driver of cardiac symptoms but points toward genetic diagnosis. ↗
▶ Ep 2 · 2:10
quote one of the hallmarks. Uh, of trisomy 13-18 is polyvacular dysplasia, which is a redundant and thickened, uh, valve. ↗
▶ Ep 2 · 4:40
epidemiological In trisomy 13, the most common lesions are patent ductus arteriosus (32%), atrial septal defects (27%), and ventricular septal defects (28%), all resulting in left-to-right shunting. ↗
▶ Ep 2 · 5:20
clinical Tetralogy of Fallot is the most common right-to-left shunt in trisomy 13, resulting in cyanosis depending on severity. ↗
▶ Ep 2 · 5:45
epidemiological In trisomy 18, ventricular septal defect is by far the most prevalent cardiac diagnosis. ↗
▶ Ep 2 · 8:40
clinical There is a very steep inflection in survival as gestational age increases for cardiac patients, with a particularly large gap between cardiac and non-cardiac patients in the 28 to 33 weeks gestation range. ↗
▶ Ep 2 · 10:00
clinical The airway is a challenging problem for cardiac surgery and can complicate liberating patients from mechanical ventilation after surgery for simple shunts. ↗
▶ Ep 2 · 10:25
clinical Pulmonary hypertension in trisomy 13/18 is unique in that it is early and accelerated in onset, which is guiding current trends in timing of cardiac surgery. ↗
▶ Ep 2 · 10:50
clinical Myocardial dysfunction seems to be more common in trisomy 13/18 patients and adds risk to cardiac interventions (institutional observation being investigated for publication). ↗
▶ Ep 2 · 14:10
clinical Some babies with trisomy 13/18 do not have the normal physiologic drop in pulmonary vascular resistance, which greatly increases surgical risk. ↗
▶ Ep 2 · 14:35
clinical For children with large VSD without trisomy 13/18, fixed pulmonary vascular resistance is not a concern until well into the first year of life, but in trisomy 13/18 this can happen much earlier in the first months. ↗
▶ Ep 2 · 14:35
quote For kids with a big VSD who don't have trisomy 13 or 18, we don't really worry about a fixed pulmonary vascular resistance problem until well into the first year of life. Uh, but this can happen much earlier in the first months, uh, in trisomy 13 and 18 ↗
▶ Ep 2 · 16:40
epidemiological Multi-center FIS database data show a clear trend of increasing numbers of cardiac surgeries being offered to trisomy 13/18 patients, with 74 centers involved. ↗
▶ Ep 2 · 17:30
epidemiological In the most contemporary multi-center dataset, only 12% of trisomy 13 and 17% of trisomy 18 patients underwent cardiac surgery. ↗
▶ Ep 2 · 17:55
epidemiological Inpatient operative survival to discharge was over 90% for both trisomy 13 and 18 in the multi-center study. ↗
▶ Ep 2 · 17:55
quote the inpatient operative survival to discharge was over 90% for both trisomy 13 and 18. ↗
▶ Ep 2 · 23:40
clinical Most PDAs in trisomy babies can be closed in the cath lab today, though some require surgical ligation based on ductal morphology. ↗
▶ Ep 2 · 26:00
clinical Ductal stenting has evolved in the last 10 years to be used widely for various forms of congenital heart disease with reduced pulmonary blood flow, including tetralogy of Fallot in trisomy patients. ↗
▶ Ep 2 · 26:30
clinical Pulmonary flow restrictors (vascular plugs with small holes) can be placed percutaneously in the cath lab to reduce left-to-right shunt symptoms as a palliative step, replacing historical use of pulmonary bands. ↗
▶ Ep 2 · 26:40
clinical Cincinnati Children's typically performs formal airway evaluation before cardiac surgery and screens for hepatoblastoma through referring centers. ↗
▶ Ep 2 · 27:00
epidemiological Since 2019, Cincinnati Children's has performed 17 index cardiopulmonary bypass procedures, mostly for simple shunts but including 6 cases of tetralogy and double outlet right ventricle, with 94% operative survival. ↗
▶ Ep 2 · 27:00
quote since 2019, we've done 17 um. Index, uh, uh, cardiopulmonary bypass, uh, procedures, um, mostly in simple shunts, but, uh, 6 of these were tetrology and double outlet right ventricle. And the operative survival at our center is right in the range of the Balt Center report at 94%. ↗
▶ Ep 2 · 28:00
quote I don't think it's all selection. I do think that selection is important, case selection. I mean, it's important for Every procedure. Right, uh, so case selection is important, but I also think that As you do more, you learn more. ↗
▶ Ep 2 · 28:00
opinion Improved outcomes are attributed to both careful case selection and institutional learning about postoperative intensive care for trisomy 13/18 patients. ↗
▶ Ep 2 · 28:30
clinical Assessment of pulmonary vascular bed status and airway status are critically important for case selection beyond just the cardiac disease. ↗
▶ Ep 2 · 29:30
clinical When the heart is enlarged, lungs are flooded, and BNP is high, cardiac catheterization is not needed before considering VSD closure. ↗
▶ Ep 2 · 29:30
quote if the heart is big and the lungs are flooded and the BMP is high, Then that's easy. We don't do a cath. We would entertain closing the hole. ↗
▶ Ep 2 · 29:55
clinical When a VSD is large and non-restrictive, there will never be a pressure gradient, so the RV will always be at systemic pressure. ↗
▶ Ep 2 · 30:15
clinical Cardiac catheterization is performed when the clinical puzzle doesn't fit together (small LV, baby not breathing fast) to assess pulmonary hypertension before surgery. ↗

Summaries James gave as host · 16 summaries

Recaps of other experts' statements, not James's own clinical position.

Summaries James gave as host · Trisomy 13 8 summaries

Open the Trisomy 13 collection →

James Cnota, MD - 2024 Fetal Care Center Navigating Perinatal Care for Trisomy 13 & 18

▶ Ep 2 · 20:00
host summary James Cnota summarizing a resource: The 2023 AATS manuscript recommends that for infants with mild to moderate complexity CHD confined to hospital, cardiac surgery is reasonable if the hemodynamic consequence prohibits discharge. ↗
▶ Ep 2 · 21:20
host summary James Cnota summarizing a resource: For infants with severe complexity and single ventricle CHD, the AATS recommendation is that it might be reasonable to not offer cardiac surgery due to anticipated poor outcomes related to pulmonary vascular disease and respiratory function. ↗
▶ Ep 2 · 21:20
host summary James Cnota summarizing a resource: infants with severe complexity and single ventricle, the recommendation is that it might be reasonable to not offer cardiac surgery. ↗
▶ Ep 2 · 22:20
host summary James Cnota summarizing a resource: For patients with mild to moderate complexity CHD who are at home or need recurrent admissions, timely elective cardiac repair is reasonable according to AATS guidelines. ↗
▶ Ep 2 · 23:10
host summary James Cnota summarizing a resource: AATS guidelines recommend that cardiac surgery involving cardiopulmonary bypass might be considered once other defects (such as esophageal atresia) are adequately addressed. ↗
▶ Ep 2 · 24:20
host summary James Cnota summarizing a resource: For ventilator-dependent patients, repair of mild to moderate complexity CHD may be considered if respiratory disease severity is not prohibitive, and long-term tracheostomy should be discussed as a possibility. ↗
▶ Ep 2 · 25:00
host summary James Cnota summarizing a resource: Because trisomy 13/18 patients are prone to early pulmonary hypertension, early cardiac surgery is reasonable in mild to moderate complex disease to prevent irreversible pulmonary vascular changes. ↗
▶ Ep 2 · 25:00
host summary James Cnota summarizing a resource: Because families and children with trisomy 13 and 18 are prone to develop early pulmonary hypertension. Early cardiac surgery is reasonable in mild to moderate complex disease ↗
Summaries James gave as host · Trisomy 18 8 summaries

Open the Trisomy 18 collection →

James Cnota, MD - 2024 Fetal Care Center Navigating Perinatal Care for Trisomy 13 & 18

▶ Ep 2 · 20:00
host summary James Cnota summarizing a resource: The 2023 AATS manuscript recommends that for infants with mild to moderate complexity CHD confined to hospital, cardiac surgery is reasonable if the hemodynamic consequence prohibits discharge. ↗
▶ Ep 2 · 21:20
host summary James Cnota summarizing a resource: For infants with severe complexity and single ventricle CHD, the AATS recommendation is that it might be reasonable to not offer cardiac surgery due to anticipated poor outcomes related to pulmonary vascular disease and respiratory function. ↗
▶ Ep 2 · 21:20
host summary James Cnota summarizing a resource: infants with severe complexity and single ventricle, the recommendation is that it might be reasonable to not offer cardiac surgery. ↗
▶ Ep 2 · 22:20
host summary James Cnota summarizing a resource: For patients with mild to moderate complexity CHD who are at home or need recurrent admissions, timely elective cardiac repair is reasonable according to AATS guidelines. ↗
▶ Ep 2 · 23:10
host summary James Cnota summarizing a resource: AATS guidelines recommend that cardiac surgery involving cardiopulmonary bypass might be considered once other defects (such as esophageal atresia) are adequately addressed. ↗
▶ Ep 2 · 24:20
host summary James Cnota summarizing a resource: For ventilator-dependent patients, repair of mild to moderate complexity CHD may be considered if respiratory disease severity is not prohibitive, and long-term tracheostomy should be discussed as a possibility. ↗
▶ Ep 2 · 25:00
host summary James Cnota summarizing a resource: Because trisomy 13/18 patients are prone to early pulmonary hypertension, early cardiac surgery is reasonable in mild to moderate complex disease to prevent irreversible pulmonary vascular changes. ↗
▶ Ep 2 · 25:00
host summary James Cnota summarizing a resource: Because families and children with trisomy 13 and 18 are prone to develop early pulmonary hypertension. Early cardiac surgery is reasonable in mild to moderate complex disease ↗