Those that would be at higher risk would obviously be those who have had significant NYHA functional class deterioration prior to pregnancy, those who have a lot of residual cyanosis, pulmonary hypertension, arrhythmias, uh, multi-organ failure, or protein losing enteropathy. These are patients that you're probably really going to classify in class 4 and advise against pregnancy.
Um, maternal deaths during pregnancy, however, are actually. Um, quite low, in fact, um, there's a manuscript sort of being, uh, worked on now that's a systematic review of multiple, um, cohort of studies that have, uh, smaller numbers, um, and in about 198 pregnancies and 110 women there are actually no deaths.
Pregnancy in a Fontan Patient: New Horizons in Medical and Surgical Fontan...
▶Ep 7 · 6:25
quoteSo this basic question to conceive or not to conceive, um, so as we discussed things so they improved survival, um, of these patients with modern technology and care, uh, these women want to know as they reach childbearing age, can I get pregnant?↗
▶Ep 7 · 7:11
clinicalNormal pregnancy causes decreased systemic vascular resistance, decreased PVR, increased heart rate, drop in blood pressure mid-pregnancy that rises again, and increase in cardiac output by about 50% and blood volume by 25%↗
▶Ep 7 · 7:24
quotethere's an increase in cardiac output by about 50% as well as an increase in blood volume by 25%.↗
▶Ep 7 · 7:33
quoteAnd then we can't forget the increased risk of thrombosis, which is about 6 times normal during pregnancy and as high as 11 times normal in the 1st 6 weeks postpartum.↗
▶Ep 7 · 7:33
epidemiologicalRisk of thrombosis is about 6 times normal during pregnancy and as high as 11 times normal in first 6 weeks postpartum↗
▶Ep 7 · 7:53
quoteSo in labor without analgesia, um, cardiac output may increase by about 30% during each contraction, so a significant pain response.↗
▶Ep 7 · 7:53
clinicalDuring labor without analgesia, cardiac output may increase by about 30% during each contraction; with regional analgesia this is mitigated to some degree↗
▶Ep 7 · 8:00
quoteWe think that with regional analgesia there is that is mitigated to some degree and so we always advocate for epidurals in our patients.↗
▶Ep 7 · 8:26
quoteIt really takes about 6 months for your cardiac output to return to non-pregnant levels postpartum.↗
▶Ep 7 · 8:26
clinicalIt takes about 6 months for cardiac output to return to non-pregnant levels postpartum↗
▶Ep 7 · 8:45
quoteSo outcomes significantly miscarriage rate is actually very high, um, among Fontan single ventricle patients, um, about 46%, um, and the neonatal death rate is also elevated.↗
▶Ep 7 · 8:45
epidemiologicalMiscarriage rate among Fontan single ventricle patients is about 46%↗
▶Ep 7 · 9:02
quoteUm, maternal deaths during pregnancy, however, are actually. Um, quite low, in fact, um, there's a manuscript sort of being, uh, worked on now that's a systematic review of multiple, um, cohort of studies that have, uh, smaller numbers, um, and in about 198 pregnancies and 110 women there are actually no deaths.↗
▶Ep 7 · 9:05
epidemiologicalIn a systematic review of about 198 pregnancies in 110 Fontan women, there were no maternal deaths↗
▶Ep 7 · 9:22
quoteSo what we are most concerned about are the morbidity side of things more than the mortality in terms of the mother, um, so SVT occurring in about 8.9%, heart failure in about 5%↗
▶Ep 7 · 9:22
epidemiologicalIn Fontan pregnancies, SVT occurs in about 8.9% and heart failure in about 5%↗
▶Ep 7 · 9:40
epidemiologicalMost Fontan women deliver between 26 and 36 weeks gestation, not making it to term (37 weeks)↗
▶Ep 7 · 9:56
quoteUh, high rates of IUGR and small for gestational age, and then the risk of, uh, congenital heart disease in the fetus is probably somewhere between 3 to 10%.↗
▶Ep 7 · 9:56
epidemiologicalRisk of congenital heart disease in fetus of Fontan mother is probably between 3-10%↗
▶Ep 7 · 10:23
quoteI just wanna specify though that, um, it actually says that significantly increased risk of maternal mortality or severe morbidity. So in this case, um, it's more the morbidity that we're concerned about, not the mortality in terms of the woman.↗
▶Ep 7 · 10:23
clinicalWHO class 3 indicates significantly increased risk of maternal mortality or severe morbidity; in Fontan pregnancy it is more the morbidity that is concerning, not the mortality↗
▶Ep 7 · 10:52
guidelineFontan women should be followed in a multidisciplinary care unit with serial echocardiograms↗
▶Ep 7 · 11:20
quoteThose that would be at higher risk would obviously be those who have had significant NYHA functional class deterioration prior to pregnancy, those who have a lot of residual cyanosis, pulmonary hypertension, arrhythmias, uh, multi-organ failure, or protein losing enteropathy. These are patients that you're probably really going to classify in class 4 and advise against pregnancy.↗
▶Ep 7 · 11:20
guidelineHigher-risk Fontan patients include those with significant NYHA functional class deterioration, residual cyanosis, pulmonary hypertension, arrhythmias, multi-organ failure, or protein-losing enteropathy; these should be classified as class 4 and advised against pregnancy↗
▶Ep 7 · 13:35
clinicalWarfarin is concerning from aspect of teratogenicity, especially in first trimester↗
▶Ep 7 · 13:46
opinionAspirin seems reasonable for most Fontan pregnant patients↗
▶Ep 7 · 13:46
quoteUm, aspirin seems reasonable for most patients, and this lady is on 162 mg.↗
▶Ep 7 · 13:51
guidelineFor Fontan patients at higher thrombotic risk (arrhythmias, prior clot, low cardiac output), therapeutic anticoagulation should be considered↗
▶Ep 7 · 14:02
quoteUm, and then if people more in the moderate range, uh, potentially considering prophylactic dose, low molecular weight heparin.↗
▶Ep 7 · 14:27
quoteUm, we absolutely feel that in this scenario delivery should be at a tertiary care center, where the highest level of cardiac OB and anesthesia and neonatal care is available↗
▶Ep 7 · 14:27
guidelineDelivery should be at tertiary care center where highest level of cardiac, OB, anesthesia, and neonatal care is available↗
▶Ep 7 · 14:50
quoteUm, it's better to have a woman on the left lateral decubitus position from the standpoint of, um, improving systemic venous return.↗
▶Ep 7 · 14:50
clinicalLeft lateral decubitus position improves systemic venous return in pregnant Fontan patients↗
▶Ep 7 · 14:56
guidelineFontan patients should be kept adequately hydrated but avoid fluid overload↗
▶Ep 7 · 14:56
quoteAnd to keep her adequately hydrated, so we, we like to, um, starve and dehydrate women in pregnancy and delivery, but in Fontan patients it's not wise to let them get, uh, too dehydrated, uh, but at the same time want to avoid fluid overload.↗
▶Ep 7 · 15:10
guidelineVaginal delivery with epidural is the preferred method for most Fontan women from cardiovascular perspective↗
▶Ep 7 · 15:10
quoteCompression stockings are a good idea and actually, uh, vaginal delivery with an epidural is the preferred method for most women from a cardiovascular perspective.↗
▶Ep 7 · 15:24
guidelineOxytocin should be used at lowest effective dose to avoid significant hemodynamic fluctuations↗
▶Ep 7 · 15:32
quoteUm, we want to continue to monitor for arrhythmias, and I would plan for these women to stay in hospital for up to about a week, um, because that's really how long it takes for things to sort of settle back out↗
▶Ep 7 · 15:32
guidelineFontan women should stay in hospital for up to about a week postpartum because that is how long it takes for hemodynamics to settle back out↗
▶Ep 7 · 15:58
guidelineEstrogen-containing contraceptives should be avoided in Fontan women because of increased thrombogenicity↗
▶Ep 7 · 15:58
quoteSo in general we avoid estrogen containing contraceptives in, in women with uh fontan circulation because of the increased thrombogenicity.↗
▶Ep 7 · 16:10
clinicalPneumoperitoneum required for laparoscopic tubal ligation may be detrimental for Fontan patients↗
▶Ep 7 · 16:19
quoteUm, so here's a table you can refer to later for some acceptable forms of contraception. I would say my favorite would be probably, um, the subdermal implants, uh, being the lowest risk just requiring, um, uh, local anesthesia.↗
▶Ep 7 · 16:23
opinionSubdermal contraceptive implants are lowest risk, requiring only local anesthesia↗
