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Hypoplastic Left Heart Syndrome
Compiled from across the library · built automatically
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
Content of this collection
Outcomes of Fontan Patients Undergoing Combined Heart - Liver Transplantation in Pediatric Hospitals Across the U.S.
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Grant Chappell, Amir Mehdizadeh-Shrifi, Darren Turner, Alexander Bondoc, Suzanne Evans, Alexander G Miethke, Gregory Tiao, Meghan M Chlebowski, Alexander R Opotowsky, David Lehenbauer, Marco Ricci, Awais Ashfaq, David L S MoralesBackground:
video0:59 · Dec 2025
Beyond 110% of nominal diameter, there is a bit of risk, but the risk is probably pretty subtle for moderate dilation (e.g., 18mm tube to 22mm) — Brian, Trans-Catheter Interventions: New Horizons in Medical and Surgical Fontan... · 15:31
Trans-Catheter Interventions: New Horizons in Medical and Surgical Fontan...
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Physicians from theCincinnati Children’s Heart Institutediscuss the long-term care of single ventricle patients from infancy to adulthood, including new insights on Fontan management and quality of life.During this session, Dr. Bryan Goldst
video17:26 · Jan 2019
Sustained Valsalva maneuvers may be particularly disadvantageous in old atrio-pulmonary Fontans due to direct effects on atrial stretch and potential for chronic atrial arrhythmias — Andrew Reddington, How Does the Blood Go Round in Single Ventricles and Fontans? New Horizons in... · 18:13
How Does the Blood Go Round in Single Ventricles and Fontans? New Horizons in...
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Physicians from theCincinnati Children’s Heart Institutediscuss the long-term care of single ventricle patients from infancy to adulthood, including new insights on Fontan management and quality of life.During this session, Dr. Andrew Redin
video24:58 · Jan 2019
Expert statements from the recordings in this collection.
Trans-Catheter Interventions: New Horizons in Medical and Surgical Fontan...
Fontan-associated plastic bronchitis occurs in less than 2% of patients with Fontan circulation
Brian1:56
Plastic bronchitis is characterized by proteinaceous lymphatic effluent into the tracheobronchial tree where there are connections between the lymphatic circulation and the airway
Brian2:01
With evaporation of water content through breathing, characteristic fibrinous airway casts remain and may be expectorated in whole or in part
Brian2:09
The incidence of fenestration thrombosis or stenosis early after the procedure and in short and mid-term follow-up is very high with bare metal stents, presumably from tissue invagination through the cells of the stent and tissue factor exposure to blood
Brian5:16
Even on anticoagulation, thrombosis is pretty common with bare metal stents used for fenestration creation
Brian5:34
Good procedures don't always mean much - one can do a good intervention in a complex population but it doesn't always mean you're going to change the ultimate clinical outcome
The Fontan population has a very high incidence of liver-related pathologies
Brian6:51
Having obstruction in the inferior limb of the Fontan can be a real problem as it only serves to augment venous hypertension
Brian7:09
A resting absence of gradient does not rule out the presence of hemodynamically meaningful obstruction
Brian7:13
Left innominate vein obstruction can create opportunity for patients to present with either plastic bronchitis or protein-losing enteropathy, two diagnoses associated with very adverse clinical outcomes that have been shown increasingly to be associated with lymphatic pathology, especially by the group from Philadelphia
Brian8:37
Secondary palliations (palliation of a palliation, the Fontan circuit) have limitations - they may not offer cure, may not even offer substantial benefits
Sometimes we're not able to test the hypothesis until we actually perform the intervention
The approach to transcatheter interventions must be taken from a multidisciplinary perspective - therapies have both medical and surgical considerations to future therapies, to the need for anticoagulation
Everything we do can have sequelae - it's really important to make sure that the multiple disciplines we use to care for these patients all understand and appreciate the implications of decision making and therapies
Stent in conduit equals thrombogenic behavior substrate
Brian12:59
A 12-year-old young man being on Coumadin is not free (not without consequences)
Having seen many of these patients grow up and face symptoms increasing through their teenage years, it's worth being aggressive when there's anatomic deficiencies within the circuit
The fluid challenge protocol involves giving 15 cc per kilo with no limit of fluid volume rapidly via central access in about 2 minutes, then starting a timer with 5 minutes from the end of fluid challenge until repeat hemodynamic runs are measured
Brian13:59
Fluid challenge is not performed if patients have overt diastolic dysfunction, defined as baseline EDP at resting EDP of greater than 15
Brian14:19
Fluid challenge is not performed if patients have baseline Fontan pressure greater than 18 and/or repeated hospitalizations with heart failure to get volume off
Brian14:30
We can safely take Gore-Tex tubes to at least 110% of their nominal diameter
Brian15:23
Beyond 110% of nominal diameter, there is a bit of risk, but the risk is probably pretty subtle for moderate dilation (e.g., 18mm tube to 22mm)
There is always risk at the anastomosis where it's hard to necessarily cover that should a problem arise because hepatic vein enters very close
Brian15:44
Gore-Tex tubes can be taken up to about 110% - the stiffness constant is very high and you cannot generate enough pressure in the balloon to stretch the Gore-Tex much more than about 110%
The risk of rupture is actually very low when dilating Gore-Tex conduits
Expert opinion16:17
There are few patients who have an undersized tube at placement and really require augmentation of the nominal diameter
Brian16:32
Extracardiac conduits are three-dimensional structures that are not circular - if a 22mm tube measures 17mm in the frontal plane, it often measures 25mm in the lateral plane, and the cross-sectional area is just fine
Brian17:01
It's important that there's a true stenosis, not just a non-circular shape, for us to want to treat conduit narrowing
How Does the Blood Go Round in Single Ventricles and Fontans? New Horizons in...
40-50% of cardiac output at baseline in Fontan patients is driven directly by the work of breathing
Andrew Reddington2:43
Negative mean airway pressure during normal ventilation draws blood into the Fontan circuit
Andrew Reddington3:14