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hypertrophic cardiomyopathy ( hcm ) is a genetic cardiovascular disease characterized by primary hypertrophy of a non - dilated ventricle .
hypertrophic obstructive cardiomyopathy ( hocm ) is generally regarded as a left ventricular ( lv ) outflow tract ( lvot ) pressure gradient ( pg ) of approximately 50 mm hg at rest or with provocative maneuvers.1 ) complications of hocm include sudden cardiac death , heart failure , and arrhythmia.2 ) in hocm patients , the cardiovascular system is exposed to acute hemodynamic burdens during pregnancy , which may lead to unfavorable complications.3 ) therefore , special monitoring and management is required for hocm patients during pregnancy .
we report on a case of a 27-year - old female patient of hocm with high lvot pg over 100 mm hg , as known as severe lvot obstruction,4 ) who maintained pregnancy and successful delivery through close monitoring using transthoracic echocardiography ( tte ) .
a 27-year - old primigravid female was referred at 9 weeks gestation for further management of known hcm .
she was diagnosed with hcm , but no further treatment was administered at that time .
she had no previous medical history , and no family history of any cardiovascular disorder or sudden cardiac death .
initially she felt palpitations rarely , but after pregnancy , she complained of exertional dyspnea and palpitation upon fast walking or overeating as 9 weeks gestation .
physical examination showed blood pressure ( bp ) of 93/64 mm hg and regular pulse rate of 108 beats per minute .
she had a grade iii / vi systolic harsh murmur at the third intercostal space on the left sternal border ( erb 's area ) .
tte showed asymmetrical hypertrophy ( maximal thickness at septum 15 mm ) with dynamic lvot obstruction due to systolic anterior motion of the mitral valve , and a severely enlarged left atrium ( volume index ; 57 ml / m ) .
hyperdynamic lv systolic function ( ejection fraction 78% ) , high lvot pg ( peak / mean pg ; 75/47 mm hg at rest , 103/52 mm hg during valsalva maneuver ) , eccentric moderate mitral regurgitation ( mr ) grade iii / iv , and pseudonormalization of lv filling pattern ( e / e ' ; 24 ) were also observed . in summary , tte showed hocm with high lvot pg and mr grade iii / iv .
initially , twenty - four - hour holter monitoring showed basically normal sinus rhythm with rare atrial premature complexes ( apc ) and ventricular premature complexes ( vpc ) . after reaching 15 weeks of pregnancy , she complained of exertional dyspnea upon fast walking and dizziness on orthostatic position change , such as standing up quickly from a seated position .
repeat tte performed at 17 weeks of gestation showed an increase in pg of 119/52 mm hg at resting and 147/70 mm hg on the valsalva maneuver .
she experienced dizziness and palpitations , which occasionally seemed to last for an entire day .
average heart rate was 91 beats per minute , faster than before , frequent apc 's ( 5568/130599 , 4.26% ) up to non - sustained atrial tachycardia and occasional vpc 's ( 522 , 0.40% ) up to triplets were checked . due to an increase of lvot pg on tte and detection of frequent arrhythmias ,
we recommended lifestyle modification including avoiding dehydration or excessive effort , and referral from a private clinic to our obstetrics department for a multidisciplinary approach .
as fetal ultrasound findings were normal , obstetricians recommended pharmacologic therapy . bisoprolol 1.25 mg twice a day was prescribed at 22 weeks of pregnancy , and the chest discomfort was improved . at 34 weeks gestation , tte showed lvot pg of 125/63 mm hg at resting and 152/72 mm hg on the valsalva maneuver ( fig .
2 ) . considering her symptoms and need for fetal growth , delivery was planned at 37 weeks of gestation .
tte performed just before delivery showed high lvot pg of 115/66 mm hg at resting and pg of 152/83 mm hg on the valsalva maneuver .
she continued taking bisoprolol until she proceeded to delivery and avoiding use of sympathomimetics or inotropics was recommended .
two days later , caesarean section with general anesthesia was performed to give a birth to a healthy baby without cardiovascular complication .
just after delivery , tte showed a decrease in lvot pg of 87/33 mm hg at rest and she was unable to perform the valsalva maneuver ( fig .
3 ) . thirteen months after delivery , a follow - up tte still showed high lvot pg of 100/31 mm hg at resting and 169/45 mm hg on the valsalva maneuver .
because of drug - refractory symptoms and severe lvot obstruction , we decided to perform an alcohol septal ablation for improving quality of life .
hcm is the most common genetic cardiovascular disorder , with prevalence of approximately 0.2% ( i.e. , 1:500 ) in the general population.5 ) this case showed how to monitor and manage a pregnancy with severe lvot obstruction ( pg over 100 mm hg ) .
pregnancy causes physiologic changes in the cardiovascular system which included increases in blood volume ( up to 40% ) and cardiac output ( 3050% ) , and reductions in systemic vascular resistance and bp.6 ) in the first and second trimesters , the increase of cardiac output is influenced by a larger stroke volume .
but later in pregnancy , a faster heart rate affects increase of cardiac output . enlarged ventricular cavity which is induced by increasing volume
reduce the lvot obstruction theoretically , however , cardiac output countervail against this effect , the lvot gradient will increase with advancing gestation.6)7 ) hcm is considered a world health organization ( who ) class ii 
iii in modified who classification of maternal cardiovascular risk , implying that depending on individual condition , there is a low to high risk of complications.8 ) cardiovascular complication rate in pregnancy with hcm appears to increase with history of previous cardiac events , poor functional class ( new york heart association iii or iv ) , severe lv systolic dysfunction , and lvot obstruction.9 ) the higher the lvot gradient is before pregnancy or during the first trimester , the higher the likelihood that symptoms will progress .
in addition , the subset of patients with severe lvot obstruction ( those with a gradient of 100 mm hg ) are at the highest risk of hemodynamic deterioration during pregnancy.4 ) pharmacologic management is recommended in symptomatic patients with hcm such as angina or dyspnea.10 ) beta blockers are mainly recommended as first - line agents because of their negative inotropic effects and decreasing adrenergic - induced tachycardia .
oral diuretics may be added with congestion symptoms.10 ) our patient showed low bp during pregnancy and a small amount of amniotic fluid was observed on fetal ultrasound at 26 weeks of gestation .
accordingly , only low dose beta blocker was administered and a modified lifestyle , avoiding aggravating factors such as excessive activity and dehydration was recommended . in our case ,
the patient had extremely high lvot pg more than 100 mm hg , resulting in a high risk of maternal cardiovascular complications .
because of her symptoms , close follow - up monitoring was performed using tte every two weeks during her third trimester . and we planned a caesarean section with consideration of maternal symptoms , fetal growth , and lvot pg on tte . as a result ,
regular tte monitoring was performed and severe cardiovascular complications and disease progression were prevented . in conclusion , we report on a case of a 27-year - old pregnant female with hocm who presented with extremely high lvot pg .
she was monitored closely with regular follow - up of tte during pregnancy to determine the timing of caesarean section , which eventually resulted in a successful delivery .
complications such as sudden cardiac death , heart failure , and arrhythmia may occur in hocm patients either throughout the course of pregnancy or during labor and delivery .
tte is a relatively safe method which does not cause harm to either the mother or the fetus .
therefore , close monitoring using tte and management with medical treatment seems essential in pregnant patients with hocm before and during pregnancy .
hypertrophic obstructive cardiomyopathy ( hocm ) patients with severe left ventricular outflow tract ( lvot ) obstruction ( those with a gradient of > 100 mm hg ) are at the highest risk of hemodynamic deterioration during pregnancy 
 . complications of hocm include sudden cardiac death , heart failure , and arrhythmias . 
 physiological changes during pregnancy may induce these complications , affecting maternal and fetal health conditions . 
 therefore , close monitoring with appropriate management is essential for the well - being of both mother and fetus . 
 we report on the case of a 27-year - old female patient with severe lvot obstruction hocm , pressure gradient ( pg ) of 125 mm hg at resting , and 152 mm hg induced by the valsalva maneuver at 34 weeks gestation . 
 this case showed how close monitoring using echocardiography and proper management during the course of pregnancy resulted in successful delivery in the patient with extremely high pg hocm .