Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation

Abstract

Background
Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs.

Case presentation
A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock–Taussig shunt at 2 years of age with transannular patching at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient’s new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation.

Conclusion
It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.

Full text

Varkolyetal. The Egyptian Heart Journal (2024) 76:46
https://doi.org/10.1186/s43044-024-00477-3
CASE REPORT
Surgically repaired tetralogy ofFallot
inthe7th decade: alate presentation ofsevere
pulmonic regurgitation
Kyle Varkoly1,5* , Akarsh Parekh2,5, Melissa Ianitelli2,5, Mostafa Hamada3, Alexandra Lucas2,4,5 and
Thomas Forbes6
Abstract
Background Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate
of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical
management in adults with TOF once pulmonary regurgitation occurs.
Case presentation A 73-year-old male with surgically corrected TOF presented with heart failure symptoms.
He underwent ToF repair with a classic right Blalock–Taussig shunt at 2 years of age with transannular patching
at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular
dilatation, and pulmonary regurgitation. Our patient’s new-onset right-sided heart failure was managed medi-
cally with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization
with resolution of symptoms and improved tricuspid regurgitation.
Conclusion It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs;
however, medical optimization should take place first. Following adequate RV load optimization, our patient under-
went successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.
Keywords Tetralogy of Fallot, Surgical repair, Seventh decade, Adult congenital heart disease, Pulmonic regurgitation,
Heart failure, Guidelines
Background
Tetralogy of Fallot (TOF) is one of the most common
cyanotic congenital heart diseases amendable to surgical
repair. e long-term results of TOF corrective surgery
and palliative shunt procedures have been promising,
with a 91% 30-year survival rate [1]. Cuyper’s single-
cohort longitudinal study in the Netherlands has data
regarding long-term cardiovascular outcomes of TOF
repair up to the 4th decade; however, data remain limited
following this period, as we are just now entering our 5th
decade post-TOF repair for many patients [2, 3]. Data
remain limited in outcomes following this decade of life.
Delayed complications following repair of TOF include
pulmonary regurgitation (PR) and right ventricular out-
flow tract (RVOT) aneurysms, both associated with right
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The Egyptian Heart
Journal
*Correspondence:
Kyle Varkoly
kylevarkoly@gmail.com
1 Department of Internal Medicine, McLaren Macomb Hospital- Michigan
State University College of Human Medicine, Mt Clemens, MI, USA
2 Department of Cardiovascular Medicine, McLaren Macomb Hospital, Mt
Clemens, MI, USA
3 College of Medicine, Kansas City University, 1750 Independence Ave,
Kansas City, MO 64106, USA
4 Center for Personalized Diagnostics, Biodesign Institute, Arizona State
University, 727 E Tyler St., Tempe, AZ 85287, USA
5 Michigan State University, East Lansing, MI, USA
6 Children’s Hospital of Michigan, Detroit Medical Center, Detroit, MI, USA

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Varkolyetal. The Egyptian Heart Journal (2024) 76:46
ventricle (RV) dilatation [4]. RVOT aneurysms are more
related to transannular patching seen post-repair [4–7].
Progressive RV volume overload from longstanding PR
results in chronic complications ranging from reduced
exercise tolerance, reduced RV and LV systolic function,
and potentially fatal atrial and ventricular tachyarrhyth-
mias [4, 6–8]. PR has been shown to be the predominant
valvular lesion prior to developing ventricular tachycar-
dia and associated sudden death. Tricuspid regurgitation
is the most common valvular lesion in patients detected
prior to developing atrial fibrillation or flutter in patients
with ToF [8].
Successfully managing the pulmonic regurgitation and
its downstream effects is of paramount importance in
this patient population. Herein we describe a case of a
patient with surgically corrected TOF in the 7th decade
of life and it is the first reported case in the medical lit-
erature with a successful outcome.
Case presentation
A 73-year-old male with the past medical history of par-
oxysmal atrial fibrillation, essential hypertension, and
surgically corrected TOF presented with worsening dysp-
nea and edema for 2weeks. He had initial palliation with
classic Blalock–Taussig–omas Shunt (BTTS) at 2years
of age rather than the aggressive surgical options avail-
able at the time, with subsequent ventricular septal defect
(VSD) repair with transannular patch placement surgery
at 18years of age. He was compliant with home medi-
cations, follow-up visits, pulmonary, and cardiac reha-
bilitations prior to hospitalization. Home medications
included Eliquis 5mg bid, hydrochlorothiazide 25mg qd,
and metoprolol tartrate 25mg bid. Physical examination
was significant for 2 + lower extremity edema, bilateral
crackles, and jugular venous distention upon arrival to
the emergency department.
Echocardiography (Fig.1) revealed a preserved left ven-
tricular ejection fraction of 60–65%, with a flattened sep-
tum in systole and diastole consistent with elevated RV
pressure and volume overload. Severe RV dilatation was
also seen with only a mildly reduced RV global systolic
function, alone with severe PR and without pulmonic ste-
nosis. Pulmonary artery systolic pressure was markedly
elevated at 108mmHg, estimated by TR jet velocity. Con-
currently, severe tricuspid regurgitation was detected on
echocardiogram.
Our patient was diuresed with IV furosemide,
which was transitioned to a daily regimen of 20 mg
daily per oral. e patient reported improved breath-
ing and decreased lower extremity swelling after diu-
resis and was then transferred to an adult congenital
heart disease center for further management of his
pulmonary valve. He underwent a right and left heart
catheterization on a separate staging catheteriza-
tion which revealed non-obstructive epicardial coro-
nary arteries, and he underwent cardiac computerized
tomography (CT) to better delineate his pulmonary
Fig. 1 Transthoracic echocardiography revealing severe pulmonic
regurgitation. a Parasternal short axis of the tricuspid valve inflow
tract showing severe tricuspid regurgitation by color flow doppler.
b Parasternal short-axis view with broad color flow jet covering
the total diameter of the right ventricular outflow tract and showing
severe pulmonic regurgitation. c Continuous-wave doppler
through the tricuspid valve showing a peak gradient of 93 mmHg
suggesting severe pulmonary hypertension in the apical view

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Varkolyetal. The Egyptian Heart Journal (2024) 76:46
arterial anatomy (Fig.2). Using a heart team approach,
he was designated as qualified for a transcatheter valve,
as he was a high-risk surgical candidate and was an
excellent fit for the Harmony™ TPV 25 (Medtronic,
Minneapolis, MN) pulmonic valve. Following appropri-
ate management of his volume overload with improve-
ment of pulmonic pressures, he was deemed ready for
pulmonic intervention.
e patient ultimately underwent transcatheter pul-
monary valve implantation with a Harmony™ TPV 25
(Medtronic, Minneapolis, MN). He tolerated the proce-
dure well with resolution of HF symptoms, undergoing
weekly pulmonary rehabilitation. He was discharged
on his home diuretic, which was discontinued at the
next office visit the following week due to concurrently
improved blood pressure with resolved edema and
dyspnea. Our patient’s tricuspid regurgitation resolved
to trace on subsequent echocardiography.
Conclusions
PR is present in most patients after anatomical correc-
tion of TOF, and its chronic impact on the right ventri-
cle (RV) often manifests dramatically, as exemplified in
our documented case. e literature has many reports
of surgically repaired TOF patients having PR in the
first few decades post-repair [4–9]. Other studies have
shown delayed onset of complications within twenty to
thirty-seven years post-repair [10, 11]. Follow-up stud-
ies, following anatomical correction of TOF repair, report
excellent long-term outcomes with a greater than eighty-
five percent survival rate up to thirty-six years post-date
of surgery at one institution in Munich, Germany [12].
However, data remain limited following the 4th decade of
repair [2]. Our case report serves as a rare and delayed
presentation of heart failure (HF), 70-year post-TOF
repair.
PR with subsequent increased RV pressures leads to
HF in patients with surgically amended TOF. PR remains
the most common valvular pathology seen before ven-
tricular tachycardia in patients with TOF, and thus inter-
ventions aimed at preserving the pulmonic valve are of
importance [6–13]. Medical management for patients
with TOF undergoing active decompensated heart failure
remains the same with preload reduction used to man-
age right-sided HF while reducing pressure and volume
in the RV.
Our patient had preserved left ventricular and right
ventricular systolic function. If either had been reduced,
it would have been a Class I recommendation for
immediate referral to an adult congenital heart disease
(ACHD) cardiologist and HF support team per 2018
American Heart Association/American College of Car-
diology Guidelines for the Management of Adults with
Congenital Heart Disease [13]. By these same guide-
lines, it is a Class I recommendation for pulmonary valve
replacement surgery given that the patient was symp-
tomatic with greater than moderate pulmonic regur-
gitation. However, given that our patient’s symptoms
improved with medical intervention, it was considered
a class I recommendation for referral to an ACHD car-
diologist with intention of subsequent workup to replace
the pulmonic valve.
Our patient had systemic congestion on the right side
of his heart, whereas the left side of his heart had sys-
temically low output. Subsequently, a systemic inflamma-
tory response was enacted which led to his presentation
involving symptoms of left-sided heart failure when sys-
temic compensatory responses declined.
Fig. 2 CT scan showing severe pulmonary arterial dilatation
indicative of pulmonary hypertension (a) with severe RV dilatation (b)

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Varkolyetal. The Egyptian Heart Journal (2024) 76:46
e timing of PV replacement surgery (rather than
intervention) is important, as one must have the replace-
ment surgery before irreversible right ventricular dys-
function takes place. Our patient had serial echos in
the outpatient setting according to ACHD guidelines,
with only asymptomatic mild pulmonic regurgitation
seen prior to this hospitalization. Given the RV dilata-
tion in our patient with greater than moderate pulmonic
regurgitation, it is a class I recommendation to plan for
PV replacement surgery given the setting of moderate–
severe PR [13]. e debate remains regarding surgical
versus interventional repair; however, with the increas-
ingly availability of transcatheter valves that fit into the
pulmonic outflow tract, the less invasive interventional
methods are increasing in popularity.
Routine follow-up testing and intervals for patients
with surgically repaired TOF among other congenital
heart diseases were also addressed in the 2018 guide-
lines. Length of time between follow-up appointments,
ECG’s, transthoracic echocardiograms (TTE’s), cardiac
magnetic resonance/cardiac computerized tomography
imaging, and exercise stress testing were addressed in
the latest 2018 AHA/ACC Guidelines for ACHD. Our
patient would be classified as stage IIc according to the
ACHD Anatomy and Physiological classification system
for moderate complexity, having prior surgically repaired
TOF with significant valvular disease. As such, he will
require increased monitoring including a visit with an
outpatient ACHD cardiologist every 6–12 months, an
ECG and TTE every year, and a CMR, CCT, and exercise
stress test every 1–2years.
rough the above interventions and use of established
guidelines, our patient is currently in the 7th decade as
his original TOF repair was at the age of 2. To our knowl-
edge, this is currently the oldest survived patient with
surgically repaired TOF in the medical literature. e
central focus of this case report lies in our deliberate
approach to a unique clinical situation. Rather than hast-
ily resorting to invasive valve replacement in response
to the patient presentation, we chose a more measured
path. We initiated treatment by administering diuretics,
a decision rooted in our recognition of the right heart’s
concomitant volume and, thus, pressure problem. e
patient’s initial presentation stemmed from chronic prior
TOF surgery, as our patient had excellent follow-up,
medical adherence, and was active in pulmonary and car-
diac rehabilitation prior to presentation.
• Outcomes past the 4th decade of life for surgically
repaired TOF have outcomes which are still under
investigation.
• Once greater than moderate PR occurs, it is a class
I recommendation for pulmonic valve intervention.
However, medical optimization should take place
first.
• Following adequate RV load optimization, our
patient underwent successful transcatheter pulmonic
valve implantation with resolution of symptoms and
cessation of diuretic.
Abbreviations
ToF Tetralogy of Fallot
PR Pulmonary regurgitation
RV Right ventricle
LV Left ventricle
Acknowledgements
Not applicable.
Author contributions
KV, MH, and AP helped in conceptualization, figure creation, writing, review-
ing, editing. MI was involved in conceptualization, figure legend assistance,
reviewing, editing, primary cardiologist. AL contributed to conceptualization,
writing, reviewing, editing. TF helped in reviewing, resources, adult congenital
heart disease cardiologist.
Funding
The authors have no sources of funding to disclose.
Availability of data and materials
All data generated or analyzed during this case report are included in this
published article [and its supplementary information files].
Declarations
Ethics approval and consent to participate
Consent was directly obtained from the patient.
Consent for publication
Written consent was directly obtained from patient.
Competing interests
The authors have no competing interests to declare.
Received: 17 January 2024 Accepted: 7 April 2024
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Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in pub-
lished maps and institutional affiliations.
Kyle Varkoly is presently in training for Internal Medicine at
McLaren Macomb, Michigan. He has a passion for academic cardiol-
ogy and was introduced early as an undergraduate at the University
of Florida with his first co-authorship investigating the relationship
between markers of inflammation in periodontal infections and
atherosclerosis. His academic interests have only continued to blos-
som with his latest papers identifying RNA viral gene signatures in
patients who developed cardiomyopathy following chemotherapy
and review articles on viral-derived biological thrombolytics. He has
continued his academic efforts through medical school at Kansas
City University and residency at McLaren Macomb, Michigan, as he
continues to pursue his passion for cardiology. His areas of interest
include coronary angiography, coronary intervention, and reducing
overall mortality and hospitalizations through the field of cardiology.
He was recently matched into cardiology fellowship at McLaren’s
flagship hospital in Flint, Michigan, beginning in July of 2024.