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Uncommon presentation of an uncommon disorder – A case series of double chambered right ventricle in adults
Congenital Heart Disease / Indian Heart Journal 69S (2017) S8–S10
by measuring aortic root, STJ and ascending aorta diameter. All patients were evaluated for aortic stenosis, aortic regurgitation, aneurysm and aortic dissection. Results: This study showed that the common type is type 2 with single raphe 76% followed by type 3 20% and type 0 rare 4%. More than 50% patients are associated with aortopathy. Two patients presented with dissection. Conclusion: We found that type 2 is the most common type of bicuspid aortic valve and more than 50% patients had aortopathy with risk of aortic aneurysm and aortic dissection. Conflicts of interest The author has none to declare. https://doi.org/10.1016/j.ihj.2017.09.021
ABS363 Acute and short term outcomes of balloon aortic valvuloplasty in patients with significant aortic stenosis – A single center experience Keyur Patel ∗ , Parminder Singh, Sharad Jain U.N. Mehta Institute of Cardiology & Research Centre, Ahmedabad, Gujarat, India
S9
ABS372 Uncommon presentation of an uncommon disorder – A case series of double chambered right ventricle in adults Kaku Singh Bhatia ∗ , Oomen K. George, Anoop George Alex, Lijo Varghese Christian Medical College Vellore, Vellore, India E-mail address: [email protected] (K.S. Bhatia). Background: Double-chambered right ventricle (DCRV) is characterized by anomalous muscle bundles that divide the right ventricle into a high pressure proximal chamber and a low-pressure distal chamber. It usually is associated with other congenital defects. It usually presents during childhood and rarely during adulthood. This series aims to document the clinical and hemodynamic profile and surgical outcomes of 9 adults with DCRV. Methodology: Nine adults (>18 years) diagnosed with DCRV based on both echocardiography and cardiac catheterization were studied retrospectively. Diagnostic information and surgical findings were collected by reviewing records. Follow up period varied from 2 to 7 years (Figs. 1 and 2). Results: The age of the patients (3 females and 6 males) varied from 18 to 48 years. Eight patients were symptomatic at presentation while 1 was incidentally detected due to the presence of
E-mail address: [email protected] (K. Patel). Background: We herewith aimed to study outcomes of balloon aortic valvuloplasty via transarterial route in children having severe aortic stenosis. Methods: A total of 58 patients with severe aortic stenosis from a super-speciality cardiac care centre were undergone balloon aortic valvuloplasty and which was successful in 56 patients. They were followed up for 6 months for assessment of their immediate and short term outcome. Results: Balloon aortic valvuloplasty remains the procedure of choice in patients with congenital aortic stenosis, particularly in paediatric age group. All patients enrolled in our study had bicuspid aortic valve morphology on echocardiography. In our study, there was statistically significant (p < 0.05) decline in transaortic gradients after BAV measured either invasively or by continuous wave Doppler method. The continuous wave Doppler derived maximum transaortic gradient which was decreased by 56.5% and mean transaortic gradient was decreased by 61% after BAV in our study. Immediate and short term outcomes were favourable as there was only 1 case with significant restenosis. 17% patients developed moderate AR and 15% patient had local complications. Conclusion: Balloon aortic valvuloplasty via trans-arterial route in paediatric population with significant aortic stenosis is safe, effective palliation for intermediate and midterm follow up with minimum complications and shown to prolong the need for early surgical intervention with very low rate of short term restenosis.
Fig. 1. Modified PSAX view showing membranous muscle bundle in the RV with turbulent flow across it.
Conflicts of interest The authors have none to declare. https://doi.org/10.1016/j.ihj.2017.09.022
Fig. 2. RV angiogram in a patient with DCRV.
S10
Congenital Heart Disease / Indian Heart Journal 69S (2017) S8–S10
Table 1 Clinical and hemodynamic profile. S. No.
Age
Gender
Presentation
Murmur
Associated anomalies
NYHA class
ECHO gdt
Cath gdt
Treatment
Post operative gdt
1 2 3 4 5 6 7 8 9
29 29 28 33 18 35 48 33 19
M F M M F M M F M
Dyspnea Dyspnea Presyncope Dyspnea None Dyspnea Chest pain Dyspnea Dyspnea
+ + + + + + − + +
Non restrictive VSD None Non restrictive VSD None Restrictive VSD Restrictive VSD None None Restrictive VSD
2 2 2 1 1 2 1 1 2
141 118 40 100 88 103 45 101 80
53 143 53 56 84 111 6 89 122
ICR ICR ICR Advised ICR-not done Medical follow up Advised ICR-not done Medical follow up ICR ICR
8 9 14 – – – – 12 7
VSD, ventricular septal defect; ICR, intra cardiac repair; ECHO gdt, echocardiographic gradient; Cath gdt, cardiac catheterization gradient.
a murmur. Dyspnea was the most common presenting symptom. Presenting symptoms varied from dyspnea, chest pain and syncope. All but one patient had audible murmur in the left parasternal border. Five patients (55%) had associated VSD. Four patients had no associated anomalies. The gradient across the anomalous muscle bundle ranged from 40 to 141 mmHg on echocardiography and from 6 to 143 mmHg on catheterization. Five patients underwent surgery, of which four had uneventful recovery. One patient developed junctional rhythm postoperatively which returned to sinus rhythm by third day. None of the patient had worsening of gradients during follow up (Table 1).
Conclusion: This is one of the largest series of DCRV during adulthood in India which highlights the atypical presentation in adults. Four of our patients had only DCRV without any associated defects which is very rare. Clinical and hemodynamic parameters were studied in detail. Treatment of DCRV cases is surgical with excellent long term outcomes. Conflicts of interest The authors have none to declare. https://doi.org/10.1016/j.ihj.2017.09.023
by measuring aortic root, STJ and ascending aorta diameter. All patients were evaluated for aortic stenosis, aortic regurgitation, aneurysm and aortic dissection. Results: This study showed that the common type is type 2 with single raphe 76% followed by type 3 20% and type 0 rare 4%. More than 50% patients are associated with aortopathy. Two patients presented with dissection. Conclusion: We found that type 2 is the most common type of bicuspid aortic valve and more than 50% patients had aortopathy with risk of aortic aneurysm and aortic dissection. Conflicts of interest The author has none to declare. https://doi.org/10.1016/j.ihj.2017.09.021
ABS363 Acute and short term outcomes of balloon aortic valvuloplasty in patients with significant aortic stenosis – A single center experience Keyur Patel ∗ , Parminder Singh, Sharad Jain U.N. Mehta Institute of Cardiology & Research Centre, Ahmedabad, Gujarat, India
S9
ABS372 Uncommon presentation of an uncommon disorder – A case series of double chambered right ventricle in adults Kaku Singh Bhatia ∗ , Oomen K. George, Anoop George Alex, Lijo Varghese Christian Medical College Vellore, Vellore, India E-mail address: [email protected] (K.S. Bhatia). Background: Double-chambered right ventricle (DCRV) is characterized by anomalous muscle bundles that divide the right ventricle into a high pressure proximal chamber and a low-pressure distal chamber. It usually is associated with other congenital defects. It usually presents during childhood and rarely during adulthood. This series aims to document the clinical and hemodynamic profile and surgical outcomes of 9 adults with DCRV. Methodology: Nine adults (>18 years) diagnosed with DCRV based on both echocardiography and cardiac catheterization were studied retrospectively. Diagnostic information and surgical findings were collected by reviewing records. Follow up period varied from 2 to 7 years (Figs. 1 and 2). Results: The age of the patients (3 females and 6 males) varied from 18 to 48 years. Eight patients were symptomatic at presentation while 1 was incidentally detected due to the presence of
E-mail address: [email protected] (K. Patel). Background: We herewith aimed to study outcomes of balloon aortic valvuloplasty via transarterial route in children having severe aortic stenosis. Methods: A total of 58 patients with severe aortic stenosis from a super-speciality cardiac care centre were undergone balloon aortic valvuloplasty and which was successful in 56 patients. They were followed up for 6 months for assessment of their immediate and short term outcome. Results: Balloon aortic valvuloplasty remains the procedure of choice in patients with congenital aortic stenosis, particularly in paediatric age group. All patients enrolled in our study had bicuspid aortic valve morphology on echocardiography. In our study, there was statistically significant (p < 0.05) decline in transaortic gradients after BAV measured either invasively or by continuous wave Doppler method. The continuous wave Doppler derived maximum transaortic gradient which was decreased by 56.5% and mean transaortic gradient was decreased by 61% after BAV in our study. Immediate and short term outcomes were favourable as there was only 1 case with significant restenosis. 17% patients developed moderate AR and 15% patient had local complications. Conclusion: Balloon aortic valvuloplasty via trans-arterial route in paediatric population with significant aortic stenosis is safe, effective palliation for intermediate and midterm follow up with minimum complications and shown to prolong the need for early surgical intervention with very low rate of short term restenosis.
Fig. 1. Modified PSAX view showing membranous muscle bundle in the RV with turbulent flow across it.
Conflicts of interest The authors have none to declare. https://doi.org/10.1016/j.ihj.2017.09.022
Fig. 2. RV angiogram in a patient with DCRV.
S10
Congenital Heart Disease / Indian Heart Journal 69S (2017) S8–S10
Table 1 Clinical and hemodynamic profile. S. No.
Age
Gender
Presentation
Murmur
Associated anomalies
NYHA class
ECHO gdt
Cath gdt
Treatment
Post operative gdt
1 2 3 4 5 6 7 8 9
29 29 28 33 18 35 48 33 19
M F M M F M M F M
Dyspnea Dyspnea Presyncope Dyspnea None Dyspnea Chest pain Dyspnea Dyspnea
+ + + + + + − + +
Non restrictive VSD None Non restrictive VSD None Restrictive VSD Restrictive VSD None None Restrictive VSD
2 2 2 1 1 2 1 1 2
141 118 40 100 88 103 45 101 80
53 143 53 56 84 111 6 89 122
ICR ICR ICR Advised ICR-not done Medical follow up Advised ICR-not done Medical follow up ICR ICR
8 9 14 – – – – 12 7
VSD, ventricular septal defect; ICR, intra cardiac repair; ECHO gdt, echocardiographic gradient; Cath gdt, cardiac catheterization gradient.
a murmur. Dyspnea was the most common presenting symptom. Presenting symptoms varied from dyspnea, chest pain and syncope. All but one patient had audible murmur in the left parasternal border. Five patients (55%) had associated VSD. Four patients had no associated anomalies. The gradient across the anomalous muscle bundle ranged from 40 to 141 mmHg on echocardiography and from 6 to 143 mmHg on catheterization. Five patients underwent surgery, of which four had uneventful recovery. One patient developed junctional rhythm postoperatively which returned to sinus rhythm by third day. None of the patient had worsening of gradients during follow up (Table 1).
Conclusion: This is one of the largest series of DCRV during adulthood in India which highlights the atypical presentation in adults. Four of our patients had only DCRV without any associated defects which is very rare. Clinical and hemodynamic parameters were studied in detail. Treatment of DCRV cases is surgical with excellent long term outcomes. Conflicts of interest The authors have none to declare. https://doi.org/10.1016/j.ihj.2017.09.023