At the beginning of the 20th century, infectious diseases were still the main cause of death in the western world. The most common infection was tuberculosis, but others also killed many, among them endocarditis. However, endocarditis was not commonly known either to physicians or to the lay public, as diagnostic tools and therapeutic measures,such as imaging, antibiotics, and cardiac surgery were not yet developed

1. Gustav Mahler’s ‘Maladie Ce´le`bre’
The life of a brilliant composer cut short by endocarditis from recurrent tonsillitis
At the beginning of the 20th century, infectious diseases were still the
main cause of death in the western world. The most common infection
was tuberculosis, but others also killed many, among them endocardi-
tis. However, endocarditis was not commonly known either to physi-
cians or to the lay public, as diagnostic tools and therapeutic measures,
such as imaging, antibiotics, and cardiac surgery were not yet
developed.
Gustav Mahler’s maladie ce´le`bre drew much attention to this so-far,
hardly known, disease. The famous composer had a fragile health
throughout his life and suffered repeatedly from many minor illnesses,
such as migraine, haemorrhoids, and recurrent throat infections. The
latter led again and again to painful Hellstein treatments of his tonsils
and not infrequently to surgical incisions of abscesses that commonly
developed.1
Mahler (1860–1912) and his Symphony No. 8, known as
‘Symphony of a Thousand’ for the great number of performers
required, vastly more than were needed for any other symphony
up to that time. Its premiere performance featured 1028 per-
formers, including an orchestra of more than 100, three choruses
and the vocal soloists. The premiere in Munich on 12 September
1910, with additional performers recruited from Vienna and
Leipzig, was greeted by a 30-min standing ovation from an audi-
ence of 3000.
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European Heart Journal (2019) 40, 3134–3142
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2. This medical history is of particular interest in the context of his last
disease several decades later. For the physicians of his time, the causal
relationship between infections of the throat with valvular heart dis-
ease developing decades later was largely unknown. In retrospect,
however, it is quite obvious that Gustav Mahler already as a boy suf-
fered from recurrent tonsillitis and this led to an immune response to
the streptococci and in turn to rheumatic fever. As a result, he devel-
oped mitral stenosis that remained asymptomatic over many years.
Indeed, Gustav Mahler was known to be an excellent hiker and did not
experience dyspnoea or any exercise intolerance during his active life.
In July 1907 his daughter, Maria, died in Maiernigg where the family
spent their summertime. Mahler asked the consulted general practi-
tioner, Dr Blumenthal, to check his heart with the stethoscope.
Dr Blumenthal heard a murmur and said to Gustav Mahler that he
should not be proud of his heart. Mahler panicked and consulted the
then famous cardiologist, Professor Kovacs, in Vienna who diagnosed a
compensated valvular heart disease; most likely mitral stenosis.
Four years later, on 20 February 1911 in New York, where he con-
ducted his VIII symphony, Mahler again suffered from his tonsillitis. He
could hardly finish the concert in Carnegie Hall and went immediately
thereafter back to his hotel to rest. He was under the impression—as
was his physician—that he had just suffered another attack of his recur-
rent tonsillitis. He took aspirin and did improve and hence his local physi-
cian diagnosed a common influenza. However, soon thereafter Mahler
collapsed after repetitive fever spikes and shivering over several days.
Thus, in panic, his physician consulted the famous New York professor,
Emmanuel Libman, who immediately diagnosed endocarditis lenta.
How was Libman able to make such a diagnosis? He was obviously a
highly educated and experienced physician and later famous for his
description of the so-called Libman–Sacks endocarditis,2
which, how-
ever, in Mahler’s case, was not the problem. Most importantly, Libman
was an astute clinician and recognized a gestalt of repetitive fevers,
shivering, skin bruises, and cardiac murmur together with a past history
of recurrent tonsillitis. He possibly also noticed an enlarged spleen and
conjunctival haemorrhages. At that time, he clearly could not have
known of the findings of vegetations on Mahler’s mitral valve.
Echocardiography had not been thought of then! Most likely, however,
he took into consideration a markedly elevated blood sedimentation
rate, anaemia, and leucocytosis in the laboratory examination.
Importantly, Libman’s assistant, Dr George Baehr, was already able to
culture from 20 mL of blood of the patient streptococcus viridans, a
haemolytic bacterium that forms strings and turns a green colour on
blood agar plates and adheres to altered surfaces of heart valves dam-
aged by rheumatic fever, a locus minoris resistentiae3
—thus, the diagno-
sis was endocarditis lenta without any doubt.
Unfortunately, at that time, Professor Libman could not go beyond
this elegant diagnosis as medicine of the day was far away for an effective
treatment of endocarditis lenta. As a consequence, the outcomes for such
patients were always fatal. Penicillin was only discovered by Alexander
Fleming many years later in 1928 and was only commonly available after
the end of the Second World War. Also, in the case of mobile vegeta-
tions, cardiac surgery to prevent embolism of the mobile vegetations
and stroke4
would not have been possible. Indeed, valvular surgery only
matured clinically as an important intervention in the 1960s.
Mahler’s fatal disease lasted for 3 months.
The composer became weaker and weaker
and crossed the ocean by ship for a second
opinion in Paris by the famous bacteriologist,
Professeur Chantemesse, at the Institute
Pasteur. Chantemesse confirmed Libman’s
diagnosis; but could not help either. A few
days later, Mahler in desperation took the
train to his hometown Vienna where he—
under continuous observation by the press—
arrived on 17 May 1911 in an increasingly deteriorating clinical condition.
Shortly, thereafter, he developed pneumonia and eventually died.
Today, endocarditis remains a dangerous disease with a high mor-
bidity and mortality in spite of the availability of sophisticated cardiac
imaging with echocardiography and 18
F-fluorodeoxyglucose positron
emission tomography,5
a growing number of effective antibiotics
against an array of bacteria, as well as cardiac surgery able to safely
remove mobile vegetations that threaten to cause embolic stroke,
drain abscesses, and replace cardiac valves destroyed by the infection.6
Andros Tofield
Docandros@bluewin.ch
Conflict of interest: none declared.
References
References are available as supplementary material at European Heart
Journal online.
doi:10.1093/eurheartj/ehz683
Pioneer in Cardiology
Alec Vahanian MD
Integrating intervention into clinical cardiology
A native of Paris, Alec Vahanian has spent most of his working life in
the city. He is best known for his expertise in valvular heart disease
and percutaneous valve interventions. He is a committed member of
the European Society of Cardiology (ESC).
CardioPulse 3135
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