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Surgery of Hypertrophic Obstructive
         Cardiomyopathy,
  Kasr El-Aini limited experience



  Elsayed K. AKL, Tarek A. Mohsen, Amal
       Khalifa and Sameh Zaghloul.
    Faculty of medicine, Cairo university
Background
    Hypertrophic obstructive cardiomyopathy
    ( HOCM) is the most common genetic
    cardiovascular disease. It affects 1:500 of
    population in different parts of the world.
    However, it did not attain enough
    attention for many years.
   There is a definite recent increasing
    interest in this disease because of the
    dramatic fatal events of sudden death in
    young patients.
Aim of work
   We sought to :
    1- Evaluate our learning curve of
    septal myectomy
    2- Investigate whether Egyptian
    patients have different phenotypic
    characteristics from other reported
    large series.
Patients and material
All patients with:
 symptomatic obstructive

  cardiomyomathy with
 maximum medical treatment and

 a resting or provoked peak systolic

  gradient > 60 mmHg. were enrolled
  in this study.
  All patients were operated by one
  surgeon .
Preoperative data

         Between Jan. 2000 & Dec. 2010
         Limited experience ( 19 patients )
                                    Age ( years )
         Sex                     2-47 (23.4 + 12.7)

 Male
63.2%
                            12         9       10
        12                  10
                             8
                             6
                             4
               7             2
                             0
                   Female
                    36.8%        < 18 y     > 18 y
Preoperative data
                  17 patients had SOB III-IV & 2 had dizziness & syncope

                   10


                    8
No. of Patients




                    6


                    4


                    2


                    0
                          0          1             2             3   4
                                         NYHA Functional Class
Preoperative data
Peak S. gradient (mmHg)                                 SWT (cm)
   60-174 (105+30)                                  1.8-3.4 (2.37+0.4)

                           Degree of MR

                       7                                          6
                       6                                5
                       5       4
     No. of Patients




                       4
                       3                      2
                       2
                       1
                       0
                           Grade 1        Grade 2   Grade 3   Grade 4
Operative Technique
   All patients underwent cautious septal myectomy
    through transaortic approach. Excision extends to the
    base of the papillary muscles.
   Attention to release of both fibrous trigones was
    added during the last 3 years.
   Septal endocardium is cautiously peeled off if
    thickened
   Only one patient required mitral repair excising
    prolapsing p1.
   Immediate postoperative peak systolic geardient was
    measured in each case by pull back or simultaneous
    left ventricular / aortic pressure tracing.
   TEE was used in most of the cases.
RESULTS ( 19 patients )

                NO op. mortality
    Immediate peak gradient dropped from a mean of
    105 + 30 to a mean of 11.5 + 6.1 mmHg.
   Significant reduction in pressure gradient was
    achieved in every patient.
    All patients came out of OR with no or trivial mitral
    incompetence.
   No incidence of iatrogenic VSD, aortic valve injury or
    complete heart block.
Examples of excised septal muscle

           P1




Thickened septal
  endocardium
Pre. & Immediate postoperative MSPG
                         Preoperative    Postoperative


  120

  100

  80

  60

  40

  20

   0
                   Mean PG
        105 + 30                        11+6.1
Pre. & postoperative PG for
                            individual patients

                                                                         Preoperative
             200                                                         Postoperative



             150
PG in mmHg




             100



              50



               0
                   1   2   3   4   5   6   7   8   9 10 11 12 13 14 15 16 17 18 19
                                               Serial Number
Post myectomy peak gradient
Pre. & postoperative MR

12                                           Preoperative
                                             Postoperative
10

 8

 6

 4

 2

 0
     Grade 0   Grade 1   Grade 2   Grade 3     Grade 4
Histopathology
Follow Up is 100%
       2 w -132 m ( mean 34.4 + 31.9)
   One patient ( 11 y ) died 3 years after operation
    out of chronic renal failure. He had family history
    of sudden death, was born with one kidney and
    had ICD 3 years before operation.
   One patient ( 35 y ) with preoperative hepato-
    renal failure died 5 weeks after operation during
    dialysyis session.
   One patient ( 16 y ) developed an attack of VT 5
    years later and treated medically.
   All survivors are well and free of SOB (FC 0-1)
Symptomatic improvement

                           Pre. & postoperative SOB
                                                          Preoperative
                  12
                                                          Postoperative
                  10
No. of Patients




                   8

                   6

                   4

                   2

                   0
                       0      1         2             3        4
                              NYHA Functional Class
Place for treatment modalities
CONCLUSIONS
   1 – Awareness of the disease and its
    consequences is mandatory.
   2 – Training with experts allows safe learning to
    properly perform septal myectomy that gives
    excellent short and long term results with
    excellent relief of gradient and symptoms.
   3 –Patients with preoperative renal problems
    seem to be at higher risk after operation.
   4- Compared to large reported series (1,2&3),
    Egyptian patients with HOCM are younger and
    present with higher gradients. This may be
    explained by particular genetic variations which
    needs to be explored.
References
1 ) Mahboob Alam, Hisham Dokanish, and Nasser M. Lakkis.
European Heart Journal (2009) 30, 1080–1087
2 ) Nicholas G. Smedira, MD, Bruce W. Lytle, MD, et al.
 Ann Thorac Surg 2008;85:127–34
3 ) J. Dearani, S. Ommen, B. Gersh et al.
 Nature Clinical Practice, cardiovascular medicine. 2007; 4 (9) : 503- 512

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Esct 18th

  • 1. Surgery of Hypertrophic Obstructive Cardiomyopathy, Kasr El-Aini limited experience Elsayed K. AKL, Tarek A. Mohsen, Amal Khalifa and Sameh Zaghloul. Faculty of medicine, Cairo university
  • 2. Background  Hypertrophic obstructive cardiomyopathy ( HOCM) is the most common genetic cardiovascular disease. It affects 1:500 of population in different parts of the world. However, it did not attain enough attention for many years.  There is a definite recent increasing interest in this disease because of the dramatic fatal events of sudden death in young patients.
  • 3.
  • 4. Aim of work  We sought to : 1- Evaluate our learning curve of septal myectomy 2- Investigate whether Egyptian patients have different phenotypic characteristics from other reported large series.
  • 5. Patients and material All patients with:  symptomatic obstructive cardiomyomathy with  maximum medical treatment and  a resting or provoked peak systolic gradient > 60 mmHg. were enrolled in this study. All patients were operated by one surgeon .
  • 6. Preoperative data Between Jan. 2000 & Dec. 2010 Limited experience ( 19 patients ) Age ( years ) Sex 2-47 (23.4 + 12.7) Male 63.2% 12 9 10 12 10 8 6 4 7 2 0 Female 36.8% < 18 y > 18 y
  • 7. Preoperative data 17 patients had SOB III-IV & 2 had dizziness & syncope 10 8 No. of Patients 6 4 2 0 0 1 2 3 4 NYHA Functional Class
  • 8. Preoperative data Peak S. gradient (mmHg) SWT (cm) 60-174 (105+30) 1.8-3.4 (2.37+0.4) Degree of MR 7 6 6 5 5 4 No. of Patients 4 3 2 2 1 0 Grade 1 Grade 2 Grade 3 Grade 4
  • 9. Operative Technique  All patients underwent cautious septal myectomy through transaortic approach. Excision extends to the base of the papillary muscles.  Attention to release of both fibrous trigones was added during the last 3 years.  Septal endocardium is cautiously peeled off if thickened  Only one patient required mitral repair excising prolapsing p1.  Immediate postoperative peak systolic geardient was measured in each case by pull back or simultaneous left ventricular / aortic pressure tracing.  TEE was used in most of the cases.
  • 10. RESULTS ( 19 patients ) NO op. mortality  Immediate peak gradient dropped from a mean of 105 + 30 to a mean of 11.5 + 6.1 mmHg.  Significant reduction in pressure gradient was achieved in every patient.  All patients came out of OR with no or trivial mitral incompetence.  No incidence of iatrogenic VSD, aortic valve injury or complete heart block.
  • 11. Examples of excised septal muscle P1 Thickened septal endocardium
  • 12. Pre. & Immediate postoperative MSPG Preoperative Postoperative 120 100 80 60 40 20 0 Mean PG 105 + 30 11+6.1
  • 13. Pre. & postoperative PG for individual patients Preoperative 200 Postoperative 150 PG in mmHg 100 50 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Serial Number
  • 14. Post myectomy peak gradient
  • 15. Pre. & postoperative MR 12 Preoperative Postoperative 10 8 6 4 2 0 Grade 0 Grade 1 Grade 2 Grade 3 Grade 4
  • 17. Follow Up is 100% 2 w -132 m ( mean 34.4 + 31.9)  One patient ( 11 y ) died 3 years after operation out of chronic renal failure. He had family history of sudden death, was born with one kidney and had ICD 3 years before operation.  One patient ( 35 y ) with preoperative hepato- renal failure died 5 weeks after operation during dialysyis session.  One patient ( 16 y ) developed an attack of VT 5 years later and treated medically.  All survivors are well and free of SOB (FC 0-1)
  • 18. Symptomatic improvement Pre. & postoperative SOB Preoperative 12 Postoperative 10 No. of Patients 8 6 4 2 0 0 1 2 3 4 NYHA Functional Class
  • 19.
  • 20. Place for treatment modalities
  • 21. CONCLUSIONS  1 – Awareness of the disease and its consequences is mandatory.  2 – Training with experts allows safe learning to properly perform septal myectomy that gives excellent short and long term results with excellent relief of gradient and symptoms.  3 –Patients with preoperative renal problems seem to be at higher risk after operation.  4- Compared to large reported series (1,2&3), Egyptian patients with HOCM are younger and present with higher gradients. This may be explained by particular genetic variations which needs to be explored.
  • 22. References 1 ) Mahboob Alam, Hisham Dokanish, and Nasser M. Lakkis. European Heart Journal (2009) 30, 1080–1087 2 ) Nicholas G. Smedira, MD, Bruce W. Lytle, MD, et al. Ann Thorac Surg 2008;85:127–34 3 ) J. Dearani, S. Ommen, B. Gersh et al. Nature Clinical Practice, cardiovascular medicine. 2007; 4 (9) : 503- 512