apical hypertrophic cardiomyopathy was first reported in 1976. Japanese scholar Sakamoto Tsugawa et al studied 9 patients with unexplained giant T wave inversion in left thoracic leads and found that There was marked asymmetric thickening of the apex. More than 40 years later, compared with classic hypertrophic cardiomyopathy (asymmetric ventricular septal hypertrophy), apical hypertrophic cardiomyopathy has not received much attention, lack of clinical research, and there is no specific guideline or expert consensus to guide clinical practice. practice.
01
pop Pathology
1) Incidence
apical hypertrophic cardiomyopathy is not uncommon, accounting for the majority of hypertrophic cardiomyopathy in Asian populations 25% of cardiomyopathy, 1%-10% in non-Asian population. Men are more common than women.
2) Ethnic factors
significantly affect the prevalence and prognosis of the disease, and Western populations have a poorer prognosis than Asian populations .
3) Family history
Positive family history is uncommon in patients with apical hypertrophic cardiomyopathy p>
02
Gene
< span>1) About 25% of patients have sarcomere gene mutations: MYBPC3, MYH7, ACTC1, MYL2, MYL3, TNNT2, TNNI3, TNNC1, TPM1.
2) Patients with positive genotype have a stronger family history.
3) There was no difference in clinical phenotype and prognosis between patients with positive and negative genotypes.
4) The European Society of Cardiology (ESC) and American College of Cardiology (ACC) guidelines for the management of hypertrophic cardiomyopathy do not recommend genetic testing for apical hypertrophic cardiomyopathy and family history screening.
Table 1: Genotypic differences between classic hypertrophic and apical hypertrophic cardiomyopathy
03
Diagnostics
1) Initially, the diagnosis of apical hypertrophic cardiomyopathy mainly relied on electrocardiogram (as shown in Figure 1) and left ventricular angiography (as shown in Figure 2).
Figure 1: Left ventricular high voltage + left chest lead T wave inversion
(especially leads V3-V5 near the apex)
04
Type
1) Simplex Apical hypertrophic cardiomyopathy: pure apical hypertrophy.
2) Mixed apical hypertrophic cardiomyopathy: apical hypertrophy + Septal hypertrophy, but mainly apical hypertrophy.
3) Occult apical hypertrophic cardiomyopathy (early apical hypertrophic cardiomyopathy):The imaging features of apical hypertrophic cardiomyopathy do not meet the diagnostic criteria (apical thickness ≥ 15 mm + ratio of apical thickness to left ventricular posterior wall thickness ≥ 1.5), these patients also have cardiac structural abnormalities: left atrium enlargement, apical ventricular wall Tumors, myocardial scars. As the disease progresses, it eventually develops overt hypertrophic cardiomyopathy (meeting the diagnostic criteria).
Figure 3: Typical ECG in occult apical hypertrophic cardiomyopathy: left ventricular high voltage + left chest Inverted
Figure 4: Cardiac MRI manifestations of occult apical hypertrophic cardiomyopathy: >Bi: relative apical hypertrophy during diastole; Bii: apical occlusion during systole; Biii: delayed apical gadolinium imaging
05
Clinical features
1) Clinical manifestations< /span>
Nearly 30%-40% of patients have no obvious symptoms, and most of them are found in physical examination< /p>
Common symptoms include chest discomfort, decreased activity tolerance, palpitations, presyncope, etc.
2) ECG performance
T wave inversion: seen in 93% of patients; 47% of patients had deep T wave inversion (>10mV)
LVH: seen in 65% of patients
3) arrhythmia
Non-sustained VT: 18% asymptomatic, 5% symptomatic
sustained VT: 3%; VT more than apical aneurysm related
AF: seen in 12% of patients; associated with left atrial enlargement, left atrial weight Structure related
4) Apical aneurysm
incidence rate: 13%-15%
Complications:apical Thrombosis, stroke, ventricular tachycardia, sudden death, heart failure
Figure 5: Mixed Apical Hypertrophic Cardiomyopathy with Ventricular Aneurysm: Diastolic Manifestations (Ai, Aii), Mid-ventricular Obstruction in Systole but Persistent Apical Space Not completely occluded (Bi,Bii), LGE imaging (Ci,Cii)
Figure 6: Simple apical hypertrophic cardiomyopathy with ventricular aneurysm: Apical thinning (Di,Dii) in diastole and apical aneurysm in systole Obvious (Ei,Eii), LGE imaging (Fi,Fii)
06
Management
1) Medication
Although β Receptor blockers (first-line therapy)/non-dihydropyridine calcium channel blockers (second-line therapy) are beneficial in classic hypertrophic obstructive cardiomyopathy, but not so far in apical hypertrophic cardiomyopathy. There is no evidence of benefit.
But beta-blockers are still the first-line treatment for apical hypertrophic cardiomyopathy because they reduce the burden of ventricular tachycardia. Non-dihydropyridine calcium channel blockers are used as second-line therapy.
Anticoagulants should be used in patients with apical aneurysms, apical thrombus, and atrial fibrillation.
2) Catheter ablation
For Apical Scar-Associated Ventricular Tachycardia
< span>3) Alcohol Ablation
If not presentAlcohol ablation is not recommended in cases of significant ventricular septal hypertrophy leading to left ventricular outflow tract obstruction
4) Apical myocardectomy
increases end-diastolic ventricular diameter and improves patient symptoms< /p>
5) Sudden Death Prevention
There are currently no predictive models to predict the risk of sudden death in apical hypertrophic cardiomyopathy to guide ICD implantation. ESC’s 5-year HCM-SCD risk score is based on all HCM patients but not apical hypertrophic cardiomyopathy, and the potential risk indicators for sudden death in apical hypertrophic cardiomyopathy (eg, apical aneurysm, paradoxical diastolic flow) are not Factors for this predictive model.
The generally accepted indications for ICD implantation are: apical aneurysm (regardless of size), cardiac magnetic resonance LGE≥15% or manifestations For diffuse, sudden death, unexplained syncope, etc.
References
References:
[1] SakamotoT, Tei C, Murayama M, Ichiyasu H, Hada Y. Giant T wave inversion as amanifestation of asymmetrical apical hypertrophy (AAH) of the leftventricle. Echocardiographic and ultrasono-cardiotomographic study.Jpn Heart J. 1976 Sep;17(5):611-29. doi: 10.1536/ihj.17.611. PMID: 136532.
[2 ] YamaguchiH, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F,Nishijo T, Umeda T, Machii K. Hypertrophic nonobstructivecardiomyopathy with giant negative T waves (apical hypertrophy):ventriculographic and echocardiographic features in 30 patients. Am JCardiol. 1979 Sep;44(3):401-12. doi: 10.1016/0002-9149(79)90388-6.PMID: 573056.
< p>[3] PaluszkiewiczJ, Krasinska B, Milting H, Gummert J, Pyda M. Apical hypertrophiccardiomyopathy: diagnosis, medical and surgical treatment. KardiochirTorakochirurgia Pol. 2018 Dec;15( 4): 246-253.d oi:10.5114/kitp.2018.80922. Epub 2018 Dec 31. PMID: 30647749; PMCID:PMC6329883.[4] HughesRK, Knott KD, Malcolmson J, Augusto JB, Mohiddin SA, Kellman P, MoonJC, Captur G. Apical Hypertrophic Cardiomyopathy: The Variant LessKnown. J Am Heart Assoc. 2020 Mar 3;9(5):e015294. doi:10.1161/JAHA.119.015294. Epub 2020 Feb 28. PMID: 32106746; PMCID:PMC7335568.< /p>
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