Hypoplastic Left Heart Syndrome
Everything in the library about hypoplastic left heart syndrome — built automatically from the recorded discussions that name it
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
Evidence & Research
1 item
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
Case-Based Learning
1 item
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
In-Depth Reviews
1 item
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
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Every expert statement below comes from the recorded discussions, with its speaker and moment.
Trans-Catheter Interventions: New Horizons in Medical and Surgical Fontan...
Fontan-associated plastic bronchitis occurs in less than 2% of patients with Fontan circulation
epidemiologicalBrian1:56 ↗
Plastic bronchitis is characterized by proteinaceous lymphatic effluent into the tracheobronchial tree where there are connections between the lymphatic circulation and the airway
clinicalBrian2:01 ↗
With evaporation of water content through breathing, characteristic fibrinous airway casts remain and may be expectorated in whole or in part
clinicalBrian2:09 ↗
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
clinicalBrian5:16 ↗
Even on anticoagulation, thrombosis is pretty common with bare metal stents used for fenestration creation
clinicalBrian5:34 ↗
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
opinionBrian5:46 ↗
The Fontan population has a very high incidence of liver-related pathologies
epidemiologicalBrian6:51 ↗
Having obstruction in the inferior limb of the Fontan can be a real problem as it only serves to augment venous hypertension
clinicalBrian7:09 ↗
A resting absence of gradient does not rule out the presence of hemodynamically meaningful obstruction
clinicalBrian7:13 ↗
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
clinicalBrian8:37 ↗
Secondary palliations (palliation of a palliation, the Fontan circuit) have limitations - they may not offer cure, may not even offer substantial benefits
opinionBrian9:47 ↗
Sometimes we're not able to test the hypothesis until we actually perform the intervention
opinionBrian9:57 ↗
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
opinionBrian10:03 ↗
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
opinionBrian10:38 ↗
Stent in conduit equals thrombogenic behavior substrate
clinicalBrian12:59 ↗
A 12-year-old young man being on Coumadin is not free (not without consequences)
opinionBrian13:05 ↗
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
opinionBrian13:18 ↗
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
clinicalBrian13:59 ↗
Fluid challenge is not performed if patients have overt diastolic dysfunction, defined as baseline EDP at resting EDP of greater than 15
clinicalBrian14:19 ↗
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
clinicalBrian14:30 ↗
We can safely take Gore-Tex tubes to at least 110% of their nominal diameter
clinicalBrian15:23 ↗
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)
opinionBrian15:31 ↗
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
clinicalBrian15:44 ↗
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%
clinical16:06 ↗
The risk of rupture is actually very low when dilating Gore-Tex conduits
opinion16:17 ↗
There are few patients who have an undersized tube at placement and really require augmentation of the nominal diameter
epidemiologicalBrian16:32 ↗
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
clinicalBrian17:01 ↗
It's important that there's a true stenosis, not just a non-circular shape, for us to want to treat conduit narrowing
opinionBrian17:19 ↗
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
clinicalAndrew Reddington2:43 ↗
Negative mean airway pressure during normal ventilation draws blood into the Fontan circuit
clinicalAndrew Reddington3:14 ↗
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