Keywords
history - congenital heart diseases - CHD - cardiovascular surgery
Introduction
Ventricular septal defect (VSD) is the most common congenital cardiac anomaly, with
an incidence of 2.62 per 1,000 live births, accounting for up to 40% of all congenital
heart defects.[1]
Surgical treatment of VSD began in 1955 by Lillehei et al using human cross-circulation[2] In Germany, the first surgical closure of VSD was performed by Professor Linder
in 1958 in Berlin.[3]
Over six decades, the surgical closure of VSD developed to be the gold standard therapy.[4]
However, patients are subjected later to clinical deterioration. We are presenting
here one of the first patients operated upon worldwide. To our knowledge, this is
the first case in literature surviving 60 years after surgical VSD closure and presenting
again with a residual VSD for cardiac surgery.
Case Description
A 68-year-old male patient presented to our clinic 60 years after surgical closure
of a perimembranous VSD at the University Hospital Berlin in 1959. The operation was
performed by Professor Fritz Linder ([Supplementary Fig. 1]) using a self-constructed heart-lung machine. The defect was 8 × 4 mm and was closed
through a right ventriculotomy with five simple sutures ([Supplementary Fig. 2]).
The patient showed postoperatively a small residual VSD, which was well tolerated
over 60 years. He played tennis regularly, and showed only a grade I dyspnea. Unfortunately,
his condition began to worsen in the last year, showing progressive dyspnea (grade
III), weakness, and progressive right heart failure (RHF).
The echocardiographic examination showed severe aortic and tricuspid regurgitation,
moderate mitral regurgitation, and normal biventricular function. The residual VSD
measured 3 mm with a small left-to-right-shunt ([Fig. 1]). The computed tomography examination showed a huge right atrium measuring 10.5 × 7 × 9.5 cm
([Fig. 2]).
Fig. 1 Transesophageal echocardiography showing (a) severe aortic valve regurgitation (VC = 7 mm), (b) moderate mitral valve regurgitation (VC = 4 mm), (c) severe tricuspid valve regurgitation (VC 17 mm), and (d) residual ventricular septal defect with left-to-right-shunt (3 mm). VC, vena contracta.
Fig. 2 Computed tomography examination of the chest with contrast showing a giant right
atrium in (a) horizontal and (b) coronal sections. LV, left ventricle; RA, right atrium; RV, right ventricle.
Significant coronary artery stenosis could be excluded through coronary angiography.
The right-sided cardiac catheterization excluded pulmonary hypertension, showing a
nonsignificant left-to-right shunt (Qp:Qs <1.5:1).
Interestingly, the patient presented to us 1 month before the operation in RHF, showing
a right-to-left shunt in the bubble study, with dilatation and marked reduction in
the right ventricular function.
The case was discussed interdisciplinary. Accordingly, the patient underwent aortic
valve replacement (Edwards Lifesciences Pericardial Bioprosthesis, 23 mm), tricuspid
valve reconstruction (annuloplasty using C-E Physio Tricuspid Ring 32 mm, and bicuspidization
of the anterior and posterior leaflets), and a primary implantation of a peripheral
venoarterial extracorporeal life support (ECLS, Maquet), to prevent potential RHF.
We decided not to close the VSD, leaving it as a vent to the right ventricle if it
would fail postoperatively, especially that it could be closed later interventionally
when indicated.
The intraoperative transesophageal echocardiography showed good function of the aortic
bioprosthesis and the tricuspid valve showed trivial residual regurgitation after
the repair.
The patient was extubated 6 hours postoperatively and the fragile unloaded right ventricle
was continuously monitored till it recovered completely after 6 days. The ECLS could
be weaned and explanted successfully. The patient could be discharged home 3 weeks
postoperatively. He received a pacemaker 3 months postoperatively due to atrioventricular
Block II and chronotropic incompetence. He could regain his activity afterwards with
only a grade I dyspnea.
Discussion
Surgical treatment of congenital heart diseases emerged in the late 1950s as one of
the first fields of cardiac surgery. With continuous development of surgical techniques,
perfusion, and perioperative management, mortality rates improved substantially over
years falling down from 30% using “Cross Circulation” to 6% within only one decade,
reaching almost 1% in the last years.[4]
The very good long-term survival made the surgical closure of VSD the gold standard
for the treatment of the indicated cases.[4]
However, patients operated upon for VSD are subjected to deterioration over the following
decades. Menting et al[5] demonstrated in their 40 years follow-up a marked deterioration over time, especially
beginning from the fourth decade. Patients with impaired systolic right ventricular
function increased from 1 to 17% in the fourth decade, with doubling of aortic regurgitation
in the past 20 years of the study period.[5] This might have been attributed to the disruption of the aortic annulus associated
with direct closure of VSD, which was the first technique used. With introduction
of patch closure technique, the risk of aortic regurgitation and residual VSD decreased
remarkably, becoming the standard technique for most cases.[6]
Chronotropic incompetence is another problem with exercise tolerance after surgical
VSD closure. This might be related to disturbances in the sinoatrial or atrioventricular
conduction system.[7]
These data are consistent with our case. The patient presented 60 years postoperatively.
Although he had a small residual VSD with a gradual dilatation of the right atrium,
and a gradual progression of his tricuspid and aortic valve regurgitation, he remained
compensated over 59 years with a minimal limitation of his physical activity. This
reflects the value of regular follow-up of patients after VSD closure, apart from
their general condition and the degree of success of their surgical repair, to be
able to intervene in the right time.
In our case, the residual VSD was a debatable issue, for which intensive discussions
among our specialists were conducted. Principally, residual shunts after surgical
closure of VSD are not uncommon, ranging from 5 to 25%, according to the type of VSD.[8]
One of the main concerns favoring closure of the residual VSD was the prophylaxis
against endocarditis. However, Gabriels et al showed in their ultra-long-term study
of 266 adult patients with VSD a 3% incidence of endocarditis in patients with nonrepaired
VSD, whereas no cases of endocarditis were identified after VSD closure, even in the
presence of a residual shunt.[9]
Other factors favoring closure were the absence of significant pulmonary hypertension
and to eliminate the shunt that might have been responsible for the other pathologies.
However, factors favoring the nonclosure of the shunt were the nonsignificance of
the shunt, the right-to-left shunt which was noticed in the decompensated condition,
the possibility to close the defect postoperatively interventionally when needed and
finally to avoid right ventricular failure if the tricuspid valve becomes competent
after the repair leaving the VSD as a ventile to the right ventricle.
Owing to the high risk of RHF postoperatively, we decided to unload the right ventricle
primarily using ECLS. We would like to emphasize the importance of the close monitoring
of the right ventricle in such cases, as it could only slowly recover, and the patient
could be weaned from ECLS after 6 days, and successfully discharged from hospital
2 weeks later.
Conclusions
Our case demonstrates the courage and persistence of our surgical grandfathers operating
under very difficult conditions; they had very limited perioperative diagnostic tools,
few medications, primitive postoperative care, and almost no monitoring. They have
used a self-constructed heart-lung-machine, and they had almost no experience regarding
correction of congenital defects. Nevertheless, they could save many lives and establish
this surgical treatment to be the gold standard over decades.
However, the presented case emphasizes the value of regular follow-up of patients
with surgically closed VSD to be able to timely identify problems and avoid possible
deterioration. Finally, in older patients with long-standing residual VSD, the primary
use of ECLS might be beneficial to prevent RHF in complex clinical situations.
