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Chronic Hypoxemic Syndrome and Congenital Heart Disease in Adults: A Multisystemic Disorder
Department of Clinic and Research Cardiology, Soma & Meintegral Clinic, Colombia
Submission: February 13, 2018; Published: May 14, 2018
*Corresponding author: John Araujo, Cardiologist-Echocardiologist in Pediatric and Adult Congenital Heart Diseases, Fellowship Integrated Unit of Congenital Heart Diseases of Adolescent and Adult Vall d’Hebron-Sant Pau, Vall d’Hebron University Hospital, Barcelona, Catalonia, Spain, Department of Clinic and Research Cardiology, Soma & Meintegral Clinic, Colombia, Tel: +5745768651; Email: email@example.com
How to cite this article: John A.Chronic Hypoxemic Syndrome and Congenital Heart Disease in Adults: A Multisystemic Disorder. J Cardiol & Cardiovasc Ther.
2018; 10(4): 555793. DOI: 10.19080/JOCCT.2018.10.555793
Congenital heart disease is the most frequent malformation, many cases are complex cyanotic congenital heart defects characterized by low arterial oxygen saturation. Unrepaired intracardiac or extracardiac congenital heart defects which causes a persistent right to left shunt, they are responsible for hypoxemic syndrome, this is a multisystemic disorder affecting various organs and symstems: hematopoietic, central nervous, gastrointestinal, urinary, cardiovascular, Immune, musculoskeletal and endocrine. The objective this review is to explain the pathophysiological mechanisms adapted to the chronic hypoxia, the consequences derived from the affectation in the different systems. The diagnostic approach and management in centers specialized in adults with congenital heart disease. This review was made with articles search sources in databases PubMed, ScienceDirect, OVID, HINARI, SciELO, MD Consult. At the end of the article review recommendations on lifestyle, exercise, travel, non-cardiac surgery, contraception and pregnancy, are given.
Congenital heart disease (CHD) in the United States (US) has an incidence of eight cases per 1,000 live births (LBs), and approximately one out of every 1,000 is complex . In Europe, the EUROCAT (European Surveillance of Congenial Anomalies) and ECEMC (Spanish Collaborative Study of Congenital Malformations) reported an incidence of 4.7 and 1.6 per 1,000 LBs, respectively. In Latin America, the RYVEMCE (Registry and Epidemiological Surveillance of External Congenital Malformations) in Mexico, and the ECLAMC (Latin American Collaborative Study of Congenital Malformations), which covers most of South America, reported an incidence of one per 1,000 and 2.3 per 1,000 LBs, respectively . Approximately 60% of CHD is diagnosed and treated early (before one year of age), and 30% during childhood and adolescence, prior to 16 years of age. An estimated 10% is diagnosed in adulthood .
Due to advances in pediatric cardiovascular surgery, currently more than 85% of children with CHD survive to adulthood . In Europe, there is an estimated population of 2.3 million adults with CHD (ACHD), which surpasses the pediatric population of 1.9 million . The ACHD population will continue to increase, with a 5-6% annual growth rate . In the US, in 2010, 1.4 million
ACHD were recorded, versus one million children with CHD. Of
the ACHD, almost 350,000 have complex defects . ACHD may be in two categories :
A. ACHD who have survived naturally
B. ACHD who received some type of therapeutic intervention (surgery or percutaneous procedure) during childhood
Adults with CHD who have survived naturally are sub classified in three groups:
a. ACHD who have required a primary therapeutic intervention during adulthood.
b. ACHD who have not required a therapeutic intervention, but need ongoing clinical supervision and follow-up.
c. ACHD who are currently inoperable, whose only intervention option is a transplant, or in whom the surgical risk is greater than the risk of natural progression.
There are congenital heart diseases with a persistent right to left flow, or shunt, cause chronic hypoxemia known as hypoxemic syndrome (HS). It commonly manifests as cyanosis.
A bluish tinge to the skin and mucous membranes which
appears when the reduced capillary hemoglobin is >5g/dl. Its
appearance depends on the amount of hemoglobin present. An
anemic patient must have a greater percentage of desaturation in
order to achieve 5g/dl of reduced hemoglobin and show cyanosis,
while cyanosis will be more intense with increased hemoglobin
levels . Cyanosis may be:
a. Peripheral: Caused by increased oxygen extraction in the
b. Central: Caused by decreased oxygen saturation in
c. Hypoxemia: Is a central cyanosis (decreased oxygen
saturation in arterial blood), caused by various disorders:
i. Cardiovascular: CHD with a right to left shunt, causing
mixing of venous or unsaturated blood with arterial blood.
ii. Pulmonary: Altered alveolar-capillary diffusion, altered
ventilation-perfusion ratio, alveolar hypoventilation.
iii. Neurological: Central alveolar hypoventilation.
iv. Environmental: Hypobaric or altitude hypoxia
v. Hemoglobin disorders: Methemoglobinemia, sulfhemoglobinemia,
An unrepaired congenital heart defect which causes a
persistent right to left shunt, mixing venous and arterial blood.
This mixing may occur in the atria (atrial septal defects), the
ventricles (ventricular septal defects), or in the vasculature
(arteriovenous fistulas, persistent ductus arteriosus). It produces
a basal arterial oxygen saturation <85% on room air (Figure 1).
Various simple or complex CHDs not repaired during childhood,
or diagnosed late in adulthood, cause hypoxemia. Some are:
Hypoxemic syndrome is a multisystemic disorder affecting
various organs. It causes serious chronic metabolic disorders
which alter the normal homeostatic equilibrium. Consequently,
the affected organs and systems function with a barely balanced
physiology in which any noxa causes serious hemodynamic
decompensation (Table 1).
Secondary erythrocytosis (SE): One of the adaptive
mechanisms to chronic hypoxemia, in the context of CCHD. It is
defined as an increase in red blood cell mass, without an increase
in all blood cell lines (polycythemia). There is a close inverse
relationship between the severity of hypoxemia, iron depletion
and the degree of erythrocytosis . The goal of SE is to increase
the supply of oxygen to the tissues, and it occurs in response to
an increased production of erythropoietin by the juxtaglomerular
apparatus in the kidney. SE produces increased blood viscosity,
which also depends on other factors such as red blood cell size,
aggregation and dispersion, plasma viscosity, temperature, and
shear stress, among others. Under normal conditions, erythrocytes
have a biconcave morphology, making them more deformable and
flexible, while the microspherocytes present in iron deficiency do
not. This explains the microcirculation problems of various organs
and sytems (kidneys, brain and lungs), producing consequences
such as in situ thromboses, kidney failure and ictus .
Hyperviscosity syndrome (HVS): A group of clinical signs
and symptoms caused by a hemodynamic disorder of slowed blood
flow resulting from increased blood viscosity. The hematocrit
level has proven to be the main determinant. Symptoms usually
appear with hemoglobin (Hgb) levels above 20mg/dl, and
hematocrits (Hct)>65%. However, this increase does not occur
in iron deficiency and microcytosis cases. Objective assessment
should always be carried out with prior iron level quantification.
There are two forms of HVS:
i. Compensated: The Hgb and Hct levels remain stable,
even with low iron levels. Hyperviscosity symptoms are
absent, or, if they are present, are mild to moderate, even with
ii. Decompensated: The Hgb and Hct levels have not been
stabilized; they remain high regardless of the presence or
absence of iron deficiency. Symptoms are always severe.
Prophylactic phlebotomy to maintain a safe and arbitrary
Hct<65%, in order to avoid hyperviscosity symptoms and cerebral
thrombotic events, is one of the principal mistaken concepts in
these patients, and is never justified. It may worsen symptoms by
reducing the minute volume, brain perfusion and pulmonary flow.
It causes iron depletion and increases hyperviscosity symptoms.
High Hct levels, in and of themselves, do not warrant repeated
phlebotomies which do not provide any clinical benefit.
There are only two clear indications for phlebotomy :
A. Moderate or severe hyperviscosity symptoms (grade 2
or 3) (Table 2).
B. Prior to surgery, if the Hct is >65%, to improve
The goal of phlebotomy is the temporary relief of
hyperviscosity symptoms, achieved by only removing the
sufficient and exact amount of blood. Clinical improvement,
evaluated by improved exercise performance, is obtained 24 hours
after the procedure. Other situations resemble hyperviscosity
symptoms: dehydration, hypovolemia, hypothyroidism, heat stroke, depression or even brain abscesses. The use of alcohol
or medications which produce systemic hypotension should be
determined, since generally these patients are medicated for
conditions other than their CHD. All these causes should be ruled
out before proceeding with phlebotomy.
Phlebotomy should not exceed 400-500ml, and should be
accompanied by simultaneous isovolumetric replacement with
nomal saline to avoid hypovolemia. The arterial pressure should
be monitored every 15 minutes and for up to one hour after
completing the procedure. If the symptoms persist, the procedure
may be repeated 24-48 hours later. Repeating the procedure more
than three to four times per year is not recommended. Plasma
or albumin infusions are not necessary. Patients with complex
unrepaired CHD with intracardiac shunting should have air-guard
filters in all their intravenous tubes, in order to avoid systemic air
emboli (Figure 2).
Anemia: Hgb levels of 15gr/dl are normal in healthy adults,
but are insufficient in patients with CCHD and Eisenmenger
syndrome. Ranges of Hgb from 16-18gr/dl are safe, to achieve
an SO2 of at least 85%, which is acceptable for the baseline
cardiopathy. Anemia is frequently not diagnosed on time. Losses
due to minor bleeds (bleeding gums, gastrointestinal bleeds,
epistaxis or metrorrhagia) are the most frequent cause. The
depletion of iron stores and low production is the second cause.
Repetitive phlebotomies are the third cause . Monitoring of
Hgb, Hct, erythrocyte indices, serum iron levels and transferrin
saturation, vitamin B12, and folic acid is recommended every
Management of Iron Deficiency: Ferritin<15mcgr/lt, and
transferrin saturation index<15%. Frequently associated with
microcytosis, they confirm serum iron depletion. Oral treatment
with 325mg/day of ferrous sulfate, or 65mg/day of elemental
iron, is recommended. Parenteral administration is indicated
if there is intolerance or a contraindication (peptic ulcer). Iron
treatment should be administered with caution, evaluating iron
stores and Hct and Hgb levels every three weeks, to avoid rebound
Hemostatic alterations: thrombosis is frequent, occurring
in the low-pressure chambers which are often dilated due to the
progression of CHD. It is facilitated by rhythm alterations (atrial
flutter and fibrillation) and prosthetic materials (valves, intra- or
extra-cardiac tubes, electrodes) which increase the formation of
thrombi. Intravascular devices (catheters, pacemakers, automatic
implantable defibrillators [AID]) cause systemic emboli. Low
levels of physical activity and sedentarism, associated with
poor peripheral venous circulation, increase the incidence
of thromboembolic events. Female sex, <85% SO2, advanced
age, ventricular dysfunction, dilation and calcification of the
pulmonary arteries, and slowed blood flow have proven to be
factors which increase the risk of thrombosis. Having an inborn
error of coagulation does not prevent thrombotic events. Laminar
thrombi in the pulmonary branches occur more frequently than
imagined (a prevalence of 38%) in the presence of dilation and
calcification of the pulmonary arteries [14,15].
Hemorrhagic diatheses: Manifest as minor bleeds (bleeding
gums, epistaxis, ecchymosis, metrorrhagia), or major bleeds
such as hemoptysis. The presence of in situ laminar pulmonary
thrombi and emboli has been documented as the cause of
massive hemoptysis. Oral anticoagulation with warfarin has
been controversial. Sandoval et al. reported no long-term
survival advantages with the use of anticoagulants in patients
with Eisenmenger syndrome and chronic cyanosis . The
severity of hemorrhagic events tends to be related to the amount
of erythrocytes and the severity of hypoxemia. As mentioned
previously, patients with significan erythrocytosis who are to
undergo surgical interventions should receive phlebotomy at
24-48 hour intervals until a hematocrit no greater than 65% or a
blood hemoglobin of 18-20g/dl is achieved, even if the patient has
no symptoms suggestive of HVS.
General recommendations in hemoptysis: Discontinue
medications that could affect coagulation, perform hemostatic
analytical studies, and take a chest x-ray. If there is alveolar
infiltrate, a chest CT scan is recommended. The patient should
be on bed rest with hydration measures, and codeine should be
administered to avoid coughing spasms. In addition, antibiotics
should be administered to avoid pulmonary superinfection.
Hypovolemia and anemia should be corrected, if present. The
possibility of administering platelets or fresh plasma should be
evaluated (platelet replacement and fresh plasma transfusion
are recommended with major bleeds and platelets <100,000/L).
When there is refractory hemoptysis, a pulmonary arteriography
should be performed, locating and embolizing the bleeding
vessel. Fibrobronchoscopy is not routinely indicated and may
produce complications, especially in patients with pulmonary
Thrombocytopenia: Platelet counts <130,000/ml have been
reported as a predictive factor of long-term mortality . Under
normal conditions, the megakaryocytes produced in the bone
marrow must travel through the circulatory bed to the lung, where
fragmentation occurs to form new platelets. In patients with CHD
and ventricular or atrial septal defects, passage through the lungs
is omitted. This causes an accumulation of megakaryocytes in the
blood and a decreased number of circulating platelets, inversely
proportional to the hematocrit and the magnitude of intracardiac
shunting. The function of existing platelets is always altered, with
reduced aggregation .
Deficiency of vitamin K dependent clotting factors:
Factors II, VII, IX, X and Factor V are deficient. There is increased
fibrinolytic activity and a deficit of the von will ebrand factor.
Clotting tests will always be altered. For a correct interpretation,
the amount of citrate used should be adjusted to the hematocrit.
Two formulas are used [19,20]:
A. The Toronto formula: One ml of anticoagulant (3.8%
citrate)=1.6 [(100-hematocrit)/100]+0.02 with a 10ml blood
B. Dr. Perloff´s formula: (100-hematocrit/595-hematocrit)
As a recommendation, the administration of oral
anticoagulants or antiplatelet agents is not warranted, since they
aggravate the hemostasis problems and worsen hemorrhages
. The exceptions to this recommendation are situations with
a clearly defined indication: sustained arrhythmias such as a trial
flutter or a trial fibrillation, a history of thromboembolism or deep
vein thrombosis, having prosthetics (catheters or intravascular
electrodes), or pregnancy . In these cases, strict control
of anticoagulation levels must be carried out in centers with
experience in managing ACHD, seeking to maintain a safe INR
range between 2-2.5.
Blood glucose levels are always low due to consumption
secondary to the elevated concentration of red cells. This must
be taken into account before carrying out unnecessary glycemic
corrections in patients.
SE and the catabolism required to replace old erythrocytes
liberate the ¨heme¨ group from hemoglobin. As a result, in order
to excrete it as a non-toxic substance, the levels of direct or
conjugated bilirubin rise, which must then be eliminated through
the bile duct. This chronic accumulation is the cause of bile stone
formation. They may cause episodes of surgical acute abdomen,
with cholecystitis attacks. They are a frequent cause of noncardiac
Secondary hyperuricemia is directly related to the presence
of heart failure, a decrease in the excretion of uric acid, and an
increase in its reabsorption, rather than to increased production
(primary hyperuricemia). Unlike what occurs in the primary
form, secondary hyperuricemia does not affect kidney function, or
affects it very little. The use of uricosuric drugs in asymptomatic
cases is not warranted. A daily dose of colchicine or allopurinol
is indicated for cases which produce true gouty arthritis. Acute
attacks may be related to the administration of diuretics which
decrease the excretion of urates. Hyperuricemia in Eisenmenger
syndrome cases is correlated as an independent long-term
mortality factor .
The deterioration of kidney function manifests with
proteinuria, decreased glomerular filtration and hyperuricemia.
Hyperviscosity increases resistance in the efferent glomerular
arteriole, hydrostatic pressure throughout the glomerulus, and
the filtration fraction, which causes an increased oncotic pressure
in the post-glomerular proximal tubule perfusion vessels, and
promotes the reabsorption of liquids and solutes (Figure 5). This
reduces the glomerular filtrate and leads to a progressive increase
in creatinine levels. There is an increased local production of
nitric oxide which causes vasodilation of the afferent and efferent
glomerular arterioles, and this is what causes the proteinuria.
Albuminuria is the first sign, which may be accompanied by
microscopic hematuria. Its incidence increases markedly
beginning in the second decade of life, and appears to be related
to the duration of cyanosis and the hematocrit levels, being a
poor prognostic marker [24-26]. Circulating megakaryocytes are
deposited in the mesangial matrix. Finally, there is ischemia and
glomerular sclerosis develops, with kidney failure.
Medications such as angiotensin converting enzyme inhibitors
(ACEI), diuretics, nonsteroidal antiinflammatory agents, and
some antibiotics should be administered with caution. Their
uncontrolled administration may trigger a kidney failure episode.
Functional cardiac alterations are inherent to the progression
of the underlying heart disease, with functional valve alterations
secondary to unrepaired structural defects (atrioventricular
septal defect and common atrioventricular valve regurgitation,
unrepaired atrial septal defect and tricuspid valve regurgitation).
Dilation of the heart chambers leads to ventricular functional
alterations which deteriorate the ventricular ejection fraction; in
consequence, various degrees of congestive heart failure appear.
Arrhythmias are a consequence of heart chamber dilation and
fibrosis in the context of a CHD. We will not specify this type of
lesions in this review.
Endothelial dysfunction manifested as a decreased
vasodilation response . Chronic hyperviscosity causes a
maximal production of nitric oxide which cannot be increased
by other external stimuli, leading to a depleted endothelial cell production of nitric oxide (NO). Cyanotic patients have an increased
terminal arteriole size and increased density of the small-calibre
vessels, and the proximal coronary arteries are dilated, ectatic
and tortuous (Figure 6). The histological changes show rupture of
the internal elastic lamina, increased collagen in the medial layer,
fibrointimal hyperplasia, and an increased extracellular matrix.
However, there is no increased risk of atherosclerosis compared
with the general population: low cholesterol levels are described
as a possible protective factor [28-30]. Ictus events are related
more to the presence of septal defects or prosthetic materials than
to the coronary artery and microcirculation changes themselves
Many times its diagnosis goes unnoticed. With the presence
of large ventricular septal defects, systemic hypertension is
transmitted to the pulmonary circuit, increasing the risk of
hemoptysis and rupture of the dilated pulmonary arteries.
Systemic antihypertensive treatment is indicated, and should be
administered with caution.
In the Eisenmenger subgroup, mortality increases 12.8 times
compared to the general population . Sudden death has been
reported at about 9%. Mortality predictors are: poor functional
class (NYHA III or IV), early age at the beginning of symptoms,
signs of heart failure, sustained arrhythmia, low albumin and
potassium levels, NT-proBNP ≥500ng/l, hyperuricemia, complex
CHD, and SO2 <85%. Echocardiographic predictors include: right
atrial area >25cm2, RA/LA area ratio >1.5, peak S wave velocity
<10cm/sec, TAPSE <15mm. Serial echocardiographic and Holter
monitor studies are recommended [33-35].
Patients with chronic hypoxemia are susceptible to viral and
bacterial respiratory infections which cause serious hemodynamic
decompensation. The bacterial germs, in order of frequency,
are: pneumococcus, staphylococcus, haemophilus, candida, and
pseudomonas . There is an increase in hard-to-treat acne, and
the risk of endocarditis caused by Propionibacterium acnes rises
in the presence of intracardiac devices . Bacterial endocarditis
is serious (many patients cannot be surgically treated due to the
severity of CHD). Yearly vaccination against influenza, and every
five years against pneumococcus, is recommended. Periodic
dental visits and assessment are advised, along with not getting
tattoos or piercings. Fingernails should be trimmed frequently
and onychophagia avoided, and soft-bristle tooth brushes are
recommended to avoid gum trauma and bacteremias [38,39].
Brain abscess is a serious complication; many times intrathecal
antibiotic administration is necessary, since the abscesses
are heavily encapsulated and poorly permeable to antibiotic
Arthralgias in knees and ankles are frequent; it occurs due
to new cellular proliferation and hypertrophic bone formation.
Periostitis is especially present in the metacarpals, metatarsals
and long bones of the forearm and legs. Nail clubbing, or drumstick
fingers, is caused by periosteal thickening, with formation of new
bone tissue throughout the diaphysis and metaphysis of the long
bones (Figure 7). Its etiology is unknown, but is presumed to be
related to hematopoietic factor stimulation in the bone marrow,
or bone growth factors which should normally be cleared in
the lungs and, due to intracardiac shunts, bypass this step .
Another complication which may be seen in adolescents and
adults is kyphoscoliosis, which may compromise pulmonary
function and aggravate the symptoms.
There is decreased exercise tolerance compared to the
healthy population, even in those with simple defects and who are
subjectively asymptomatic. During exercise, there is peripheral
vasodilation, with decreased systemic resistance, leading to an increased venous-arterial shunt (right to left) through the
anatomical heart defects, with increased arterial hypoxemia,
hypocapnia and acidosis. Rapid muscle fatigue and tiredness
are the consequences. In addition, there is an altered ventilatory
dynamic with elevation of the ventilation equivalent (ventilation
volume per unit of CO2 produced by exercise; VE/VCO2). A stress
test with oxygen may be helpful in evaluating the degree of
desaturation triggered by exercise [41-43].
Neuroendocrine neoplasms are described in ACHD and
chronic hypoxemia. The origin lies in the mitotic cellular alteration
and differentiation of cells in very metabolically active tissues
(glands). Pheocromocytomas, paragangliomas, ganglioneuromas
and neuroblastomas have been described [44,45], with increased
symptomatology, fluid imbalance and general cardiovascular
hemodynamics alterations. Alexander et al. described 18 cases of
pheocromocytomas and paragangliomas, in a multicenter study.
Most of the patients had cardiovascular or psychiatric symptoms.
The average length of cyanosis was 20 years (range 1-57). The
mean age at diagnosis was 31.5 (15-57 years) and seven cases
were multiple tumors. Hospitalized patients with CCHD had
a higher probability of tumors (adjusted odds ratio 6.0, 95%
confidence interval 2.6-13.7, P<.0001) than those without CHD;
patients with non-cyanotic CHD did not have a greater risk (odds
ratio 0.9, p=0.48) .
Hemodynamic anesthetic changes such as decreased
systemic vascular resistance, increased pulmonary pressure and
myocardial depression increase the right to left shunt, leading to
more hypoxemia. The loss of volume due to bleeding drastically
alters the hemodynamic status with serious decompensation
and increased mortality, especially in patients with Eisenmenger
syndrome. If these surgeries are strictly necessary and urgent,
they should be performed in specialized centers with a
multidisciplinary team and a cardiologist specialized in ACHD.
Epidural anesthesia causes sympathetic block, affecting preload
and post-load. This causes a serious alteration in the
hemodynamic state and the risk of death. General anesthesia can
be better controlled. Venous lines with air filters should always
be used (Figure 2). Bacterial endocarditis prophylaxis should be
provided and HS recommendations followed. Arrhythmias and
thromboembolic events are frequent, so preventive measures
should be taken [47-50].
The pressurized air in airplanes simulates an altitude of about
1,800-2,400 meters above sea level. Most patients tolerate it well.
The gradual ascent of the airplane produces Hgb dissociation
curve similar to that of the healthy population. Supplemental
oxygen is not necessary during the flight. Airplane cabins are
warmer and have less humidity, which favors the loss of fluids
and dehydration. Drinking sufficient liquid is recommended,
along with not drinking alcoholic beverages or ingesting sedative
or anxiolytic medications on long trips (they trigger serious
hemodynamic instability and the risk of sudden death). If the
destination is at a higher altitude (to that at which the patient
usually lives), portable oxygen is recommended when the patient
disembarks, as well as having a means of transportation available,
in the event of decompensation. Peripheral edemas or headaches
have rarely been reported during flights .
Prophylactic measures are recommended to avoid deep vein
thrombosis (walking, moving legs, choosing aisle seats). Also, the
trip should be prepared well in advance and emotional stress
avoided. For long trips, additional necessary recommendations
should be coordinated with the cabin crew and the ACHD
Long road trips require the same recommendations. If the
destination is over 1, 500 meters above sea level, the ascent
should be gradual, avoiding rapid climbs which can cause
decompensation (visits to mountain or natural park overlooks).
These climbs should be carried out with caution and portable
oxygen is recommended. Heights over 2,500 meters above sea
level are not recommended. Anti-emetics should be administered
with caution; they can cause sedation, hypotension, collapse and
hemodynamic decompensation .
Avoid isometric exercises (muscle building) or intense
effort or competitive activities. Poor oxygenation and muscle
metabolism cause rapid fatigue and loss of muscle strength
(increased accidents), as well as a high risk of sudden death
caused by arrhythmias or a fall in systemic output. Swimming
in deep water should be avoided (drowning and gas embolism
accidents). All contact sports are contraindicated (risk of major
bleeding due to trauma). Long, hot showers or saunas should
be taken with caution. Mechanical rides and high jumps are
Pregnancy is contraindicated (mortality >50% in Eisenmenger
cases). Complications occur especially in the third trimester
and the first post-partum days. Only 15-25% of pregnancies
get to term, and 50% have intrauterine growth restriction and
prematurity . Increased levels of progestins decrease the
systemic vascular resistance, increasing the right to left shunt,
which causes severe cyanosis. Vasovagal stimuli or minor bleeds
are a cause of sudden death in the Eisenmenger subgroup .
Barrier contraception is not effective; potentiation with the use of
spermicides is recommended. In the event of a failure, emergency
contraception is recommended. Combined oral contraceptives
(OCs) are contraindicated (increased thromboembolic risk). Pure
progestin OCs is recommended. Subdermal implants are four times more effective than surgical sterilization, but they only last
for three years, and the local anesthesia used for implantation is
well tolerated .
Progesterone intrauterine devices are effective and safe, longterm;
the only risks during implantation are a vasovagal reaction,
minor risk of infection, and scant irregular bleeding during the
first months after implantation . Surgical tubal ligation guided
by laparoscopy, or the implantation of intratube coils guided by
hysteroscopy, is effective. They imply an anesthetic risk, and
should only be performed at specialized centers .
Chronic hypoxemic syndrome is a multisystemic disorder.
The clinician must not only know the pathophysiology and
hemodynamic behavior of the CHD itself, but must also
understand the pathophysiological changes that occur in all the
organs and systems, paying attention to the early diagnosis of
frequent complications. The natural evolution of an adult with
CHS oscillates between asymptomatic periods and others with
clinical deterioration and the potential risk of death. Management
should be undertaken by a multidisciplinary team headed by
a cardiologist expert in the care and management of ACHD.
Management errors lead to increased morbidity and mortality;
therefore, timely follow-up in specialized centers is pertinent.